MicroRNA Levels in Exhaled Breath Condensate of Patients with Esophageal Atresia.

MicroRNAs (miRNAs) are noncoding RNAs that play an important role in the regulation of inflammation and have not been evaluated in exhaled breath condensates (EBC) of patients with esophageal atresia and tracheoesophageal fistula (EA-TEF). It is aimed to evaluate the levels of miRNA-21 and miRNA-24 in EBC of patients with EA-TEF. Patients who received surgery for EA-TEF (EA) were assessed for age, sex, types of anomaly, surgical treatments, and respiratory problems. A 500-1000 mL of EBC was obtained from each participant with EcoScreen. The levels of miRNA-21 and miRNA-24 in the EBC were analyzed by real-time polymerase chain reaction and compared between the EA group and the control group consisting of healthy children with no history of respiratory problems (n = 17). The levels of miRNAs in relation to respiratory problems and gastroesophageal reflux (GER) were also assessed. A total of 19 patients were enrolled in the EA group with a mean age of 7.8 ± 3.2 years and a male-to-female ratio of 10:9 EA cases had significantly lower levels of miRNA-21 (P < 0.05) compared to that in control group. The miRNA-24 levels did not differ between groups (P > 0.05). EA patients with positive pH testing for GER (n = 6) and fundoplication (n = 6) had higher levels of miRNA-21 than those with normal pH testing and without fundoplication, respectively (n = 13, P < 0.05). The levels of miRNA-21 and miRNA-24 did not differ between patients with and without proton pump inhibitor treatment (P > 0.05). The lower levels of miRNA-21 in the EBC of EA patients suggest a hyperreactive airway problem, which may be associated with GER and its surgical treatment.

Management of bilateral Wilms tumor over three decades: The perspective of a single center.

INTRODUCTION: Outcomes of Wilms tumor improved in last 50 years and excellent survival rates can be achieved especially in case of non-metastatic disease and favorable histology. Nevertheless, bilateral cases still stand as a therapeutic challenge. Prognosis of bilateral Wilms tumor (BWT) is not as good as the unilateral tumors of similar stage and histology in terms of survival and renal function. OBJECTIVE: Management of BWT is constantly evolving and still stands as a therapeutic challenge. This study is designed to review and share our experiences on this topic from a surgical standpoint. STUDY DESIGN: The records of patients treated in our clinic between 1980 and 2013 according to Turkish Pediatric Group of Oncology protocol were analyzed retrospectively and clinical data, surgical details, pathology results, long term outcomes were analyzed. RESULTS: Thirteen girls and 7 boys with a mean age of 2,5 years were treated. There were 2 patients with Wilms tumor-Aniridia-Growth Retardation complex and one with isolated hemihypertrophy. Metastasis were detected in lungs of 4 patients; liver of 2 and in the cranium of one. All patients except one with the presumptive diagnosis of unilateral Wilms tumor were given preoperative chemotherapy. To sum up; 19 nephroureterectomies, 8 partial nephrectomies and 13 enucleations were performed to 36 kidneys without any major early or late postoperative complications. Pathologic results revealed positive surgical margins in 2 lesions with enucleation and in 2 with partial nephrectomies and anaplasia in 4 patients. Two patients were not operated due to parental disapproval. Two patients had the need of dialysis; one was anephric and the others' renal functions recovered over a year. Seven patients received radiotherapy for pulmonary metastasis, positive surgical margins or local recurrences. Overall, 13 patients survived and 7 died due to metastasis, recurrences, and complication of dialysis and refusal of surgical treatment. Survival among all patients was 65% and 72.2% among operated ones. Of the 7 patients with the partial nephrectomy, 2 died and 5 survived. Among enucleation group, 8 out of 10 survived and 2 died. Survival was slightly higher among enucleation group (80% vs 71.4%). Median time of follow-up for survivors of disease is 5.8 years (min: 6 months and max: 14 years). DISCUSSION: Outcomes of BWT management have changed dramatically during the last few decades from only survival, to a long life expectancy without the need of renal replacement therapy owing to improvements in treatment options. We argue that positive surgical margins do not necessarily lead to local recurrence. For this reason it may be wiser to favor on more nephron sparing surgery than to achieve negative surgical margins. Adjuvant chemotherapy and radiotherapy may be adequate to prevent local recurrence. Also, survival did not differ significantly between different ways of nephron sparing surgeries, so it may be wiser to choose enucleation over partial nephrectomy which preserves more nephrons. Nephron-sparing surgery should have utmost importance despite the risk of positive margins. On the other hand, there is not enough data to interpret if positive surgical margins have role on distant metastases or not. Presence of metastasis and recurrence seems to be an important determinant of prognosis given the fact that none of the survivors had any metastasis or recurrence. CONCLUSION: Nephron preservation should be the aim while taking positive surgical margin risk on nephron sparing surgery side relying on postoperative chemotherapy and carefully planned radiotherapy to avoid recurrence. However, there is significant diversity on the management BWT in different centers and a certain validated guideline or protocol to provide the optimal treatment is still lacking.

Transient hypertrophic pyloric stenosis due to prostoglandin infusion.

Prostaglandin E1 (PGE1) is widely used in ductus-dependant congenital heart disease to maintain the patency of ductus. Hypertrophic pyloric stenosis (HPS) due to gastric mucosal proliferation is a rare complication of prolonged PGE infusion. A male newborn who developed HPS during PGE1 infusion is presented to discuss the clinical features and treatment modalities of PGE-related transient HPS. The boy was 2500 g and born at 35 weeks of gestation from a 23-year-old mother. He was admitted to neonatal intensive care with breathing difficulty and cyanosis. His echocardiography revealed pulmonary atresia, ventricular septal defect and major aorta-pulmonary collateral (MAPCA). PGE infusion with a dose of 0.05 mcg kg⁻¹ was initiated. At the 8th day of infusion, he developed non-billous vomiting. Ultrasonographic evaluation revealed 1.9 cm length of pyloric channel and 0.5 cm of wall thickness on 11th day and diagnosed as HPS. On 42th postnatal day, he underwent MAPCA closure, right modified Blalock-Taussi shunt and repair of pulmonary artery bifurcation with bovine patch. PGE infusion was stopped and enteral nutrition was started on 8th postoperative day. Control ultrasonography on 12th postoperative day revealed normal pyloric channel length (0.9 cm) and wall thickness (0.3 cm). Prolonged use of PGE infusion in neonates with congenital heart disease may cause transient HPS. The clinical and radiological features of HPS relieves after stopping PGE infusion. It should be kept in mind that HPS due to PGE infusion can be transient and pyloromyotomy should be kept for patients with persistent findings.

Heart rate variability analysis reveals a shift in autonomic balance towards an increase in parasympathetic tonus in boys with undescended testis.

AIM: Evaluations of sacs and cremaster muscles associated with undescended testis (UT) have revealed a decrease in sympathetic, but an increase in parasympathetic tonuses. Since the low frequency/high frequency power ratio obtained through spectral analysis of heart rate variability (HRV) determines the sympatho-parasympathetic balance, the sympatho-parasympathetic balance as defined by HRV in healthy boys and in boys with UT were compared. MATERIAL AND METHODS: Sixteen healthy boys and 21 boys with UT presenting as an isolated anomaly were evaluated. Powers in low and high frequency bands were determined by spectral analysis, and low frequency/high frequency ratios were calculated. Data were compared by analysis of variance, and p-values less than 0.05 were considered significant. RESULTS: Age distribution in the groups was similar. Low frequency/high frequency ratios were 2.27+/-1.84 and 1.26+/-0.79 in healthy boys and in boys with UT, respectively (p=0.0087). CONCLUSION: The autonomic balance is shifted with a decrease in sympathetic and an increase of parasympathetic tonus in boys with UT. This difference in signaling may help to understand the decrease in fertility, and the increase in the risk of malignancy in men with UT.

Clinical presentation and management of gastroesophageal reflux disease in a referral center in Turkey.

PURPOSE: The aim of this study was to analyze the characteristics of the pathway from the onset of clinical findings related to gastroesophageal reflux disease (GERD) until the surgical therapy to achieve a better organization of the multiple disciplines and create the best management scheme in a referral center in Turkey. PATIENTS AND METHODS: All patients who underwent anti-reflux surgery for GERD in our unit between 2000 - 2006 were retrospectively reviewed. Information on their past medical follow-up, the clinical findings, diagnostic evaluation and the therapeutic approach was recorded. RESULTS: There were 24 girls and 40 boys with a median age of 36 months (3 - 192 months). Of the 64 cases, 36 (56.3 %) had a history of past medical follow-up. Of these 36 patients, 20 had received medical and/or surgical therapy for GERD without any standardization. The remaining 16 were treated supportively for neurological, respiratory and metabolic problems without having had a diagnosis of GERD. The median duration of symptoms in 28 patients without a past medical history was shorter than that in the remaining 36 patients (p = 0.03). Of the 64 patients at presentation, 35 had neurological, 4 had metabolic, and 3 had a respiratory pathology; the other 4 had a history of esophageal atresia and tracheoesophageal fistula operation; 5 had been operated in another center for GERD and only 13 cases had isolated GERD. The most commonly used diagnostic methods were contrast study (n = 52) and pH monitoring (n = 36). Forty-one of the 64 had anti-reflux surgery primarily after presentation, in addition to selection of medical therapies and/or other surgical interventions as a first step in the remaining 23. Reevaluation of those 23 patients after a median period of 7 months (1 - 36) finally led to anti-reflux surgery (ARS). Patients presenting with a stricture due to GERD (n = 13) underwent ARS, either as a primary procedure (n = 3) or after a course of dilatations (median period of time: 8.5 months) (n = 10). CONCLUSIONS: Extended nonresponsive medical therapy is as harmful as needless surgical therapy performed prior to appropriate medical management. Our experience emphasizes that guidelines on the use of a multidisciplinary approach is the first step for successful GERD treatment. ARS in early infancy should only be justified in the presence of severe neurological and/or respiratory pathologies. If there is any doubt about the diagnosis of GERD with preliminary methods, endoscopic and pathological confirmation of the disease is mandatory for a correct management.

The use of pH monitoring and esophageal manometry in the evaluation of results of surgical therapy for gastroesophageal reflux disease.

BACKGROUND/PURPOSE: Surgery is indicated for the treatment of gastroesophageal reflux disease (GERD) when medical treatment fails or complications are encountered in children. However, it has not been fully established how the results after surgery can be evaluated. A prospective study was performed to evaluate the results of surgical therapy for GERD by pH monitoring (PM) and esophageal manometry (EM) in children. METHODS: Patients who were candidates for anti-reflux surgery between 2003 and 2004 were evaluated for symptoms, growth and results of PM and EM both in the pre- and postoperative periods. RESULTS: Thirteen patients were included (mean age = 6.65 +/- 3.28 years, male/female ratio = 10/3). Most frequently occurring symptoms were recurrent respiratory infections (RRI) (n = 11) and vomiting (n = 8). Nissen fundoplication was performed because of unresponsiveness to treatment (n = 10), RRI (n = 9), failure to thrive (n = 7) and esophagitis (n = 2) after medical treatment (2 - 36 months). Symptoms were resolved in 83.9 % of patients and were not changed in 16.1 % following surgery. Weight percentiles had significantly improved (pre: 12.38, post: 25.4, p < 0.05) during a short follow-up period (1 - 4 months). Mean reflux index (pre: 24.73 +/- 21.07 %, post: 0.93 %, min: 0 - max: 3.6, p < 0.05), reflux time (pre: 368 +/- 313 min, post: 17.1 +/- 15.9 min, p < 0.05), number of episodes (pre: 344.7 +/- 343.6, post: 19.53 +/- 11.13, p < 0.05) and number of reflux episodes longer than 5 minutes (pre: 4.3, min: 0 - max: 58, post: 0.61, min: 0 - max: 3, p < 0.05) were found to be reduced after surgery by PM. Lower esophageal sphincter pressure (pre: 55 +/- 27 cmH (2)O, post: 64.46 +/- 30.85 cmH (2)O), contraction amplitude (pre: 141.92 +/- 69.11 cmH (2)O, post: 130.69 +/- 45 cmH (2)O) and contraction velocity (pre: 1.94 cm/s, min: 0.1 - max: 7.5, post: 4.29 cm/s, min: 0.2 - max: 10) did not differ postoperatively (p > 0.05). However, contraction times were decreased postoperatively (pre: 73.6 +/- 52.9 s, post: 27.67 +/- 20.1 s, p < 0.05) and were found to be correlated with reflux time and the number of reflux episodes longer than 5 minutes. CONCLUSION: Nissen fundoplication is effective for the treatment of GERD. It supports the anti-reflux mechanism without affecting esophageal motility except for contraction times. The decrease in contraction time after surgery can be explained by the decreases in reflux time and in the number of reflux episodes longer than 5 minutes. PM and EM confirmed the clinical improvement and can be used for the evaluation of results of NF.

Sarco(endo)plasmic reticulum and plasmalemmal Ca(2+)-ATPase activities in cremaster muscles and sacs differ according to the associated inguinal pathology.

Sarco(endo)plasmic reticulum Ca(2+)-ATPase (SERCA) and plasmalemmal Ca(2+)-ATPase (PMCA) activities in cremaster muscles and sacs, which have been subjected to different autonomic tonuses, were determined and compared. Samples of cremaster muscles and sacs associated with male or female inguinal hernia, hydrocele or undescended testis were obtained from children during operations and activities of SERCA and PMCA were determined. While highest SERCA and PMCA activities were encountered among cremaster muscles and sacs associated with undescended testis, least activities were encountered among structures associated with hydrocele. The alterations in SERCA and PMCA activities in cremaster muscles associated with undescended testis appear to reflect the attempts at maintaining the levels of cytosolic calcium. Despite similar total calcium contents, lower SERCA and PMCA activities were found in sacs associated with hydrocele compared to those associated with undescended testis suggest a difference among the levels of cytosolic calcium.

Sigmoid colon vaginoplasty in children.

BACKGROUND: Vaginal construction is necessary for the patients with aplasia of Mullerian ducts, testicular feminisation and androgen insensitivity syndromes. Many methods of vaginal construction have been described. We report here the outcomes of six adolescent patients who underwent sigmoid colon vaginoplasty with special emphasis on the surgical technique and outcomes. PATIENTS AND METHODS: Between 1990 and 2003, six patients underwent sigmoid vaginoplasty after a diagnosis of 5alpha-reductase deficiency (n = 3), testicular feminisation (n = 2) or vaginal atresia (n = 1). The mean age was 16 years (13 to 18). Wide spectrum antibiotics and whole-gut preparation were used in all cases. A 15-20 cm segment of sigmoid colon was pulled through the retrovesical tunnel. The proximal end was closed in two layers in patients with 5alpha-reductase deficiency and with testicular feminisation. A distal anastomosis was carried out to the opening made on the vaginal plate (5alpha-reductase deficiency) or on the tip of the shallow rudimentary vagina (testicular feminisation). The sigmoid segment was interposed between the blind end of the atretic vagina and the perineum in the patient with vaginal atresia. Patients were instructed to perform daily vaginal irrigation. The neovagina was examined and calibrated under anaesthesia. No routine vaginal dilatation was recommended. RESULTS: All but one patient had an uneventful postoperative period and were discharged within 7-8 days. All patients had an excellent cosmetic result with an appropriate vaginal length. One of the patients experienced late stenosis of the introitus which responded to dilatations. Mucus discharge was not a significant problem. The patient with vaginal atresia (Bardet-Biedl syndrome) experienced deep vein thrombosis, renal failure and sepsis, resulting in death. CONCLUSION: Sigmoid colon vaginoplasty is a special procedure which appears appropriate for the construction of a new vagina in children. A sigmoid colon neovagina meets all necessary criteria after a vaginoplasty. It provides an adequate diameter and length, and produces less scar tissue in the perineum. It is self-moistening, easily adaptable to the uterus, cervix and rudimentary atretic vaginal segments and does not require routine dilatation. Mild stenosis of the introitus can be treated by dilatations and revision can be easily performed in severely stenotic cases. On the other hand, the patient may face morbidity after laparotomy and other serious complications may occur due to accompanying diseases.

Bannayan-riley-ruvalcaba syndrome from the point of view of the pediatric surgeon.

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is an overgrowth syndrome and polyposis condition, which carries an increased risk for development of benign and malignant tumors of various tissues and organs. A 9-year-old boy with BRRS who had multiple subcutaneous mesenchymal tumors and follicular adenoma of the thyroid and a 14-year-old boy with multiple subcutaneous mesenchymal tumors and gastrointestinal polyposis are presented, with special emphasis on the surgical management of these patients.

Anti-androgen induced inhibition of testicular descent is associated with a decrease in sympathetic tonus.

BACKGROUND/AIMS: Responses of cremaster muscles and sacs from boys with undescended testis suggested less exposure against sympathetic, but more exposure against parasympathetic tonuses. Since the sympathetic tonus is androgen dependent, it has been suggested that androgens control the descent by influencing the sympathetic tonus. Therefore an experimental study was planned to evaluate the contractile responses of cremaster muscles according to locations of associated testes in rats subjected to intrauterine steroidal or non-steroidal anti-androgen exposure. METHODS: Time-mated pregnancies were started in 18 rats. They were divided into three groups and each group was given physiologic saline, flutamide or cyproterone acetate (Androcur Depot). Flutamide was administered from day 15 to day 19, cyproterone acetate on day 15 of intra-uterine life. At twelve weeks of age the localization of testes was evaluated, cremaster muscles were removed, and contractile properties were studied. Twitch and tetanic contractions were recorded isometrically at 37 degrees C. Effects of verapamil and isoprenaline were investigated. Results were compared by ANOVA and p values less than 0.05 were considered significant. RESULTS: Both testes of all available male offspring in the saline (n = 22) and cyproterone acetate-treated (n = 19) groups were in the scrotum. Sixty percent of males in the flutamide-treated group (n = 20) had undescended testes. Cremaster muscles of rats exposed to flutamide had a lower sensitivity to voltage sensitive Ca+2 channel blockade by verapamil (3 x 10(-4) mol/L, p < 0.05) and displayed greater contractile response to isoprenaline (10(-5) mol/L, p < 0.05). Alterations in contractile properties of the muscles did not differ according to localization of testes in rats subjected to flutamide exposure. CONCLUSIONS: CM in rats subjected to non-steroidal anti-androgen exposure revealed alterations that indicate a decrease in sympathetic tonus. Since non-steroidal anti-androgen also inhibits the descent, the present study provides experimental support for the involvement of sympathetic tonus in the androgenic control of testicular descent.

Myogenesis within the human gubernaculum: histological and immunohistochemical evaluation.

BACKGROUND: The presence of varying amounts of smooth muscle (SM) in the patent processus vaginalis suggests that SM plays a role in the descent of the testis. Myogenesis within the gubernaculum (representing primitive mesenchymal tissue) has been evaluated. MATERIALS AND METHODS: Bilateral gubernacula of ten male and five female fetuses were obtained. Sections were stained with hematoxylin-eosin, van Gieson and Gomori trichrome. Expressions of human muscle actin, desmin, vimentin, alpha-smooth muscle actin, human myosin, fast myosin, slow myosin, and Myo-D were determined through immunohistochemistry. The structural alterations and expressions according to the fetal ages were determined within the gubernacula of both sexes. RESULTS: Gubernacula revealed striated muscle at twelve weeks of age. Despite expression of actin and desmin, those muscles did not express Myo-D. Actin and desmin expressing striated muscles ceased to exist until 22 weeks of age. Both vascular SM and cremaster muscle (CM) expressed Myo-D during the 22nd and 23rd weeks. CM additionally expressed alpha-smooth muscle actin. Detection of myofibroblasts in the 22nd week was followed by appearance of the SM in the 27th week in the gubernacula of male fetuses. The same alterations were encountered among the female fetuses at later time points. CONCLUSION: Both smooth muscle and CM develop within the gubernaculum. CM may have transdifferentiated from the vascular SM. If the gubernaculum represents primitive mesenchymal tissue, it ceases to exist after the development of muscles.

The effects of orchidopexy and orchidectomy on testes of rats subjected to ipsilateral abdominal testis with vas deferens obstruction.

An experimental study was planned to evaluate and compare the effects of orchidopexy and orchidectomy on the testes of rats subjected to unilateral abdominal testis with vas deferens obstruction. Four groups were established. Rats in the control group underwent a sham procedure. While the testis was maintained in the abdomen with the vas deferens ligated for 8 weeks in group 2, rats in groups 3 and 4 underwent orchidopexy or orchidectomy after 4 weeks. Remaining testes were harvested at the end of the 8-week period. Testis and body weights were obtained during harvest. Samples were evaluated through DNA flow cytometry, and percentages of haploid cells were determined. Groups were compared through unpaired t-test, and p-values less than 0.05 were considered significant. All three treatments had decreased testis weight over body weight values of ipsilateral testes. Ipsilateral orchidectomy increased the value among contralateral testes. However, none of the groups had a contralateral testicular value less than the sham-operated group. All three treatments decreased the percentage of haploid cells among ipsilateral testes, but only an abdominal testis was associated with a decrease in the percentage of haploid cells among contralateral testes. Maintaining a testis with an obstructed vas deferens in the abdomen for 8 weeks damages both ipsilateral and contralateral testes. Orchidopexy, while showing amelioration of the ipsilateral testis, spares the contralateral testis as well as orchidectomy. Orchidopexy for an undescended testis with vas deferens obstruction is a rational approach.

Thymic teratoma or thymic remnant attached to mediastinal teratoma? The cellular origin of mediastinal teratomas revisited.

Thymic tissue can be encountered in about 20 % of mediastinal teratomas. It is found located at the periphery of the teratoma mass. Although rare, mediastinal teratoma arising within the thymus can be seen. It has not been defined yet whether the thymus is an organ simply attached to the teratoma or whether the teratoma arises from the thymus. The authors present here two illustrative cases of mediastinal teratoma, one raised within the thymus and the other closely attached to the thymic remnant, to discuss the cellular origin of mediastinal teratomas.

Autonomic nervous system appears to play a role in obliteration of processus vaginalis.

BACKGROUND: Failed obliteration of processus vaginalis (PV) has recently been proposed to be due to persistence of the smooth muscle (SM), which is transiently present to generate the physical force to descend the testis. Sacs from hernia, hydrocele, or undescended testis reveal alterations in Ca(2+) contents. Since Ca(2+) signaling and some regulatory proteins are involved in programmed cell death (PCD), a flow cytometric evaluation was planned to evaluate the expression of inducers or inhibitors of PCD in sacs from different diagnostic sources. METHODS: Sacs associated with male hernia ( n=16), female hernia ( n=11), undescended testis ( n=9), and hydrocele ( n=11) were evaluated for binding of annexin and expressions of Bcl-2, Bax, Fas, Fas-ligand (Fas-L), and Fas+Fas-L. The binding and expressions in cells that express alpha-smooth muscle actin (alpha-SMA) were also determined. Expressions according to the groups were compared through unpaired t-test, and P values less than 0.05 were considered to be significant. RESULTS: Sacs associated with undescended testis that contain the least SM revealed the most annexin binding, and sacs associated with hernia that contain the most SM binded the least annexin. However, expressions of Bcl-2, Bax, Fas, Fas-L and Fas+Fas-L have not revealed a significant difference. On the other hand, Bax and Fas expressions of cells that express alpha-SMA have been significantly higher in sacs associated with undescended testis. CONCLUSIONS: Since increase of Bax and Fas in cells that express alpha-SMA are encountered in sacs with the least SM content, Bax and Fas appear to play roles in the amount of persisting SM. The necessities of initial depletion of Ca(2+) stores through inositol 1,4,5-trisphosphate receptors and subsequent increase of Bax and Fas levels in the mitochondrial pathway of PCD, together with the role of the sympathetic system in maintenance of SM, suggest a determinative role of the autonomic nervous system for obliteration of PV.

Sclerotherapy for lymphangioma in children.

Although surgical excision has been considered to be the treatment of choice by most of the surgeons, sclerotherapy of lymphangioma has gained popularity during recent years. A prospective clinical trial was conducted to evaluate the efficacy of bleomycin and OK-432 sclerotherapies for treating lymphangioma in children. Fifteen patients were enrolled in the study conducted between 1998 and 2002. All patients were hospitalized. Bleomycin or OK-432 was injected into the lesion. Patients were observed in the hospital for 1 day. The response was considered as excellent in cases with total disappearance of the lesion. If the lesion was regressed more than 50% of the original size, the response was considered as good. Little or no change in the size of lesion was considered to be a poor response. Ten girls and five boys with ages ranging from 4 days to 12 years were treated. Five patients had been operated previously for lymphangioma. Most of the lesions were located only in the cervical region (n=13). Other cases revealed extensions into the mediastinum (n=1) or axilla (n=1). Bleomycin (n=8, 2.87+/-2.03 inj.), OK-432 (n=5, 2+/-1 inj.) or both in order (n=2, 6 and 16 injs.) were injected. No allergic reaction, scar formation or pulmonary complication was encountered. Fever (11%), local reactions (4%) and vomiting (2%) were encountered following a total of 55 injections. After a follow-up period of 6-36 months, the responses were excellent in 53.4%, good in 26.7% and poor in 6.6%. Macrocysts disappeared in the remaining 13.3% of patients who had mixed cervicofascial lymphangioma. Lower success rates were encountered among patients who had undergone prior surgery. Sclerotherapy with bleomycin and OK-432 is effective in the treatment of lymphangioma in children. Adverse effects are minor and rarely encountered. However, the surgeon should be alert for the possible serious complications. Sclerotherapy can be also used as an adjunctive therapy in the treatment plan of widespread or mixed forms of lymphangiomas. In the light of our results, sclerotherapy should be preferred as the primary mode of treatment in childhood lymphangiomas.

Pulmonary metastases in children: an analysis of surgical spectrum.

Pulmonary surgery is frequently used for the treatment of metastasis or nodules in children with various types of malignancies. However, the indications and effectiveness of pulmonary metastatectomy have not been evaluated recently. Therefore, a retrospective study was conducted to analyse the results of pulmonary metastatectomy in children. Children who underwent pulmonary metastatectomy at our department between 1990 and 2000 were reviewed. Eighteen children consisting of 11 boys and 7 girls (age range, 3 to 18 years) underwent thoracotomy for pulmonary metastasis excision. The primaries were osteosarcoma (n = 2), synovial sarcoma (n = 1), fibrosarcoma (n = 1), Ewing's sarcoma (n = 2), mesenchymal chondrosarcoma (n = 1), Wilms' tumour (n = 4), clear-cell sarcoma (n = 1), Hodgkin lymphoma (n = 3), hepatoblastoma (n = 1), hepatocellular carcinoma (n = 1) and haemangioendotheliosarcoma (n = 1). Pulmonary metastases were encountered either at the time of initial diagnosis (22 %) or occurred within 6 months to 5 years. They were frequently nodular (94 %), unilateral (94 %) and located in the right lung (70 %). The number of metastases were frequently one (56 %) or two (28 %). Excision was done by means of wedge resection (88 %), segmentectomy (6 %), and lobectomy + wedge resection (6 %). The nodules contained tumour cells in most cases (n = 14) (78 %), mature nephrogenic elements (6 %) and no tumour tissue (16 %) in the remaining cases. Histology was similar to that of the original tumour in 12 cases. However, synovial sarcoma was encountered in metastasis in one case with fibrosarcoma primary. Re-thoracotomy was performed in 22 % of cases for the recurrent lesion, which in only one case was a true local recurrence. Overall disease-free survival rate was 56 % during the follow-up period (mean, 36.4 +/- 31.8 months). Pulmonary metastatectomy may increase survival in carefully selected children, though it is unlikely to cure the patient. Therefore combined therapies such as chemotherapy and/or radiotherapy should be continued in the postoperative period.

Xanthogranulomatous pyelonephritis in children: diagnostic and therapeutic aspects.

BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is an uncommon chronic, inflammatory disease of the kidney. Etio-pathogenesis, diagnosis and management of pediatric XGP is still obscure due to the limited number of cases. Therefore, a retrospective clinical study was carried out to present an updated picture of the entire spectrum of pediatric XGP based on our 30 years' experience covering one of the largest non-collected series treated in a single medical center. METHODS: Records of children who were treated for XGP in our unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical history, clinical characteristics, diagnostic procedures,treatment methods, histopathologic findings and outcome. RESULTS: 17 children with a mean age of 6.6 +/- 0.8 years, consisting of 15 males and 2 females, were treated for XGP. Eight patients had a urological disease history and 4 of them underwent surgery for urinary calculi and exstrophia vesica repair. The most common presenting symptoms were abdominal pain, fever,weight loss and anorexia. Palpable flank mass was the most common physical examination finding. Left and right kidneys were involved in 10 and 7 patients, respectively. Diagnostic procedures were intravenous pyelography, retrograde pyelography, ultrasound scan and computerized tomography (CT). Renal calculi and/or calcifications and non-functioning kidney were the most frequent findings in radiologic investigations. By combining all these investigations, the preoperative diagnoses were XGP (n = 2), renal and/or perirenal abscess (n = 5), psoas abscess(n = 2), non-functioning kidney due to recurrent pyelonephritis(n = 4), Wilms' tumor and/or renal clear-cell carcinoma (n = 4). XGP was correctly diagnosed in only two patients based on cr findings. Drainage of the renal or psoas abscess was performed in 8 patients as an initial procedure (surgical drainage in 6 and ultrasound-guided percutaneous drainage in 2 patients). Surgical procedures included nephrectomy (n= 13), partial nephrectomy (n=2), nephrectomy and diversion of reno-colic fistula(n= 1), and renal biopsy and nephrostomy (n= 1). Operative and postoperative complications were colonic perforation (n= 3) and wound infection (n = 3). Complications were noted only in patients who underwent nephrectomy without initial drainage procedures. Histopathologic examinations showed diffuse and focal XGP in 14 and 3 patients, respectively. CONCLUSION: XGP should be included in the differential diagnosis of all children presenting with perirenal or psoas abscess, renal mass and/or non-functioning kidney associated with/or without urolithiasis. Clinical awareness and a high index of suspicion is required to achieve the correct preoperative diagnosis and appropriate management. CT seems to be the most valuable imaging method for the diagnosis. We strongly recommend percutaneous drainage of the abscess and adjunctive antibiotic therapy prior to nephrectomy to avoid complications. Complete nephrectomy is the proper treatment for the diffuse form whereas frozen section biopsies followed by partial nephrectomy are mandatory for the proper treatment of focal disease.

Colonic atresia: surgical management and outcome.

Colonic atresia (CA) is a very rare cause of intestinal obstruction, and little information has been available about the management and predictors of outcome. A retrospective clinical trial was performed to delineate the clinical characteristics of CA with special emphasis on surgical treatment and factors affecting outcome. Children with CA who were treated in our department between 1977 and 1998 were reviewed: 14 boys and 4 girls aged 1 day to 5 months. All but 2 referred patients and 1 with prenatal diagnosis presented with intestinal obstruction. Plain abdominal X-ray films showed findings of intestinal obstruction in 14 cases; a barium enema demonstrated a distal atretic segment and microcolon in 4. The types of atresia were IIIa (n=9), I (n=6), and II (n=3). Type IIIa atresias were located proximal to the splenic flexure (n=8) and in the sigmoid colon (n=1), type I atresias were encountered throughout the colon; and all type II atresias were proximal to the hepatic flexure. Associated anomalies were multiple small-intestinal atresias (MSIA) (n=4), gastroschisis (GS) (n=2), pyloric atresia (n=1), Hirschsprung's disease (n=1), and complex urologic abnormalities (n=1). The initial management was an enterostomy in 15 patients (83%), including 2 referred and 2 with GS, and primary anastomosis in the remaining 3 (17%). Secondary procedures were the Santulli operation (n=2), colostomy closure and recolostomy followed by a Swenson operation (n=1), sacroabdominoperineal pull-through (n=1), and colostomy closure (n=1). Leakage was encountered in all primarily anastomosed patients. The overall mortality was 61%. Deaths occurred in patients with associated major anomalies (GS 2, MSIA 3, pyloric atresia 1) (55%) and in 3 patients who were initially managed by primary anastomosis (27%). Two additional patients died of sudden infant death syndrome (18%). Type I atresia was more common than in previously reported series and was associated with proximal multiple atresias. The initial management of CA should be prompt decompression of the intestine by an ostomy procedure, preferably end- or double-barrel. The type of surgery (primary anastomosis without prior colostomy) and associated abnormalities are the major determinants of poor outcome.

Testicular tumors in children.

PURPOSE: The aim of this study was to present an updated picture of surgical management of pediatric testicular tumors based on our 30 years' experience, which consisted of one of the largest noncollected series treated in a single medical center. METHODS: Records of children who were treated for testicular tumor in our unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical history, clinical characteristics, diagnostic procedures, treatment methods, histopathologic findings, and outcome. RESULTS: Fifty-one patients with a mean age of 3.8 +/- 0.5 years were treated for testicular tumors. Of these, 35 (69%) had germ cell testis tumor (GCT) and 16 (31%) had non-germ cell testis tumor (NGCT). Endodermal sinus tumor and paratesticular rhabdomyosarcoma were the dominant histologic subtypes in each group, respectively. The most common mode of presentation was painless scrotal mass. At initial presentation, retroperitoneal (n = 5), both retroperitoneal and lung (n = 2), and retroperitoneal and liver (n = 3) metastases were recorded in 10 (19%) patients. Initial operative procedures were radical inguinal orchiectomy (RIO) (n = 29), scrotal orchiectomy (SO; n = 9), bilateral RIO (n = 2), both RIO and unilateral retroperitoneal lymph node (RPLN) excision (n = 6), testis-sparing enucleation of the tumor (n = 5). SOs were performed elsewhere, and these patients underwent high ligation (n = 4) and both high ligation plus RPLN excision (n = 5) in our unit. Histopathologically, spermatic cord invasion and RPLN involvement were present in 10 patients. Scrotal recurrences were encountered in 2 patients who had scrotal orchiectomy initially. Retroperitoneal recurrences were noted in a patient presenting with stage I embryonal carcinoma and in 2 patients presenting with group IV paratesticular rhabdomyosarcoma. The mean follow-up period was 89 +/- 10 months. Four patients with stage IV embryonal carcinoma (n = 2) and group IV paratesticular rhabdomyosarcoma (n = 2) died of progression of the disease. All remaining patients were alive and disease free at their last outpatient appointment. No significant difference was noted with regard to 5-year survival rates between (1) malignant GCT and paratesticular rhabdomyosarcoma patients (91% v 80%) and (2) patients treated by RIO (88%), SO plus high ligation (87%), and RIO plus RPLN excision (80%). Five-year survival rates were 100% for stage I, II, III patients and 33.3% for stage IV and group IV patients presenting with malignant testicular tumors (P <.05). CONCLUSIONS: Childhood testicular tumors deserve special attention from the therapeutic point of the view. A solid scrotal mass should be considered malignant until proved otherwise. Any suspicion of the testicular tumor warrants an inguinal approach to prevent scrotal violation by the tumor. Current trends emphasize that testis-sparing surgery should be performed for benign lesions such as teratoma, leydig cell tumor, and epidermoid cyst based on frozen biopsy findings. Literature findings and our experience suggest that RIO is the accurate treatment for stage I malignant GCT and group I and IIa paratesticular rhabdomyosarcoma. RPLN excision is not of benefit either as a staging or therapeutic procedure in stage I and group I and IIa diseases of these tumors. RPLN excision should be reserved for (1) malignant GCT patients who have persistent elevation of alpha-fetoprotein after orchiectomy in the presence of normal total body CT scan, and for patients presenting with stage II and III disease with definitive abnormality on CT scans, and (2) group IIb, IIc, and III paratesticular rhabdomyosarcoma patients with radiologic evidence of retroperitoneal involvement on CT scans. High ligation should be done as a complementary procedure after SO to increase the survival rates. J Pediatr Surg 36:1796-1801.