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melikoglu, mustafa/0000-0001-9646-3787; Aydin, Burca/0000-0002-6013-6271 WOS: 000278808800001 PubMed: 20367260 Aim: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. Methods and patients: Between 1998 and 2006, WT patients were registered from 19 centers. Patients 2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. Conclusions: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

OBJECTIVE AND METHOD The survival of the patients with neuroblastoma has improved in last few decades. But still it depends on various clinical and biological factors. To assess the clinical features and trends in survival, the data for 500 newly diagnosed patients between January 1972 and December 2004 from a single center were retrospectively analyzed. RESULTS Histopathologic subtypes were neuroblastoma (NBL) in 462 patients (92.4%) and ganglioneuroblastoma in 38 patients (7.6%). The median age was 2.9 years and Male/Female ratio was 1.3/1. Primary tumor sites were abdomen, thorax, pelvis, neck, and others with the frequency of 72.2%, 14.9%, 3.8%, 3.2%, and 5.9%, respectively. There were 30, 49, 133, 257, 31 patients with stage 1, 2, 3, 4, 4S disease and their 10-year survival rates were 100%, 75.8%, 34.1%, 6.5%, and 59.4%, respectively. The outcome has significantly improved according to 10-year periods. The 5-year overall survival rates were 14%, 26.1%, 39.2%, and 52.4% for the years of 1970s, 1980s, 1990s, and after 2000. Surgical procedure involving total or near total tumor removal improved the survival (P=0.002). Both 5-year overall survival and event free survival rates were higher when partial resection was performed, especially in stage 3 disease (P=0.002 and P=0.02). In multivariate analysis, age above 18 months at diagnosis (P=0.01), stage 4 disease (P

Progressive familial intrahepatic cholestasis (PFIC) is a cholestatic liver disease of childhood. Pruritus secondary to increased bile salts in the serum may not respond to medical treatment. Partial external biliary diversion (PEBD), which reduces the serum bile salt level in the enterohepatic cycle, is used in the treatment of this symptom. In this study, our experience in performing this technique and the early promising results of PEBD in two children with PFIC are reported along with a review of the current literature. Partial external biliary diversion was performed by interposing a 15-cm jejunum between the gallbladder and abdominal wall. Biliary drainage through a stoma began in the fi rst postoperative day and reached 120-200 ml/day. Pruritus improved and then stopped on the 15th postoperative day, while the serum bile acid concentration also decreased. Partial external biliary diversion by jejunal interposition provides an excellent control of pruritus in children with PFIC with no adverse effects. A cholecystectomy should therefore be avoided in patients with PFIC.

Ingestion of a foreign body (FB) is a prevalent condition among children. The type of FB varies according to the feeding habits and sociocultural features of communities. The management modality differs also between disciplines due to use of conventional techniques. We aimed to picture the general characteristics of FB ingestion and treatment alternatives, to mention the indications of open surgery in an advanced pediatric surgical center. The records of patients who were hospitalized for FB ingestion between 1973 and May 2005 were evaluated retrospectively. One hundred and twelve patients were enrolled into the study. The mean age was 2.27 +/- 2.84 years with a M/F ratio of 59/53. The history was suggestive of ingestion in 92% of patients. The age did not differ significantly whether the history was positive or negative (3.6 years vs. 4.8 years and P = 0.19). Most common presenting symptom was vomiting (28.6%). The duration of symptoms was longer in patients with negative history (median 47.7 h vs. 28.1 h and P < 0.002). Physical examination was normal in 89.3% of cases. Most common localization of the FB shown in plain X-ray was the esophagus (67%). Esophagography revealed nonopaque FB in the esophagus in 4.4%. X-ray was normal in 6.3% of the patients. The age of patient did not determine the localization of FB on admission (P = 0.436). Endoscopic removal was attempted in 75% and was successful in 68% of patients in which FB was extracted by using laryngoscope and Magill forceps (12%), rigid esophagoscope with FB forceps (51%), and flexible endoscope with FB forceps (5%). FB could not be found in 32% of patients at initial rigid esophagoscopy or flexible endoscopy. FB was eliminated spontaneously (n = 19) or extracted surgically (n = 8). Follow-up was preferred in 21% of patients on initial admission. FB proceeded uneventfully in 15 patients or was extracted by flexible endoscopy or surgery in one and eight patients, respectively. Surgery was performed in 4% on admission. Surgery or endoscopy were essentially required in cases whose follow-up period exceeded 4 days when compared with patients who eliminated FB spontaneously within 4 days, independent to the location of FB. The metallic objects were the frequently ingested FBs (83.8%) in which the safety pins (SPs) (n = 53) and coins (n = 25) were the most frequent. The type of FB did not affect the FB localization on admission (P = 0.38). The duration of hospitalization was longer in patients with delayed admission; 2.46 +/- 3.51, 3.80 +/- 8.17, and 5.72 +/- 4.24 days for the admissions within first, second-fifth days, and sixth or later days after ingestion, respectively (P = 0.000). Pediatric surgery has the largest spectrum of duty in the treatment of FB ingestion in children. Negative history, normal physical examination findings and absence of symptoms do not exclude the possibility of FB ingestion. Presentation with isolated respiratory symptoms is an enigma that can lead to misdiagnoses. The mode of management should be selected according to the patient's condition, surgeon's experience, and available technical equipment as well as the location and type of ingested FB. Especially, SPs should be treated by experienced surgeons. Simple extraction techniques and both rigid and flexible endoscopies with appropriate forceps as well as surgery can be used for the extraction of FBs lodged in the alimentary tract. Surgery can be expected especially in asymptomatic cases that have been followed up for more than 4 days irrespective of the location of FB.

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.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.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, p0.05) 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, p0.05), reflux time (pre: 368 +/- 313 min, post: 17.1 +/- 15.9 min, p0.05), number of episodes (pre: 344.7 +/- 343.6, post: 19.53 +/- 11.13, p0.05) and number of reflux episodes longer than 5 minutes (pre: 4.3, min: 0 - max: 58, post: 0.61, min: 0 - max: 3, p0.05) 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 (p0.05). However, contraction times were decreased postoperatively (pre: 73.6 +/- 52.9 s, post: 27.67 +/- 20.1 s, p0.05) and were found to be correlated with reflux time and the number of reflux episodes longer than 5 minutes.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.

Background/Purpose A retrospective study was performed to evaluate risk factors, clinical features, and treatment modalities of portal vein thrombosis (PVT) after splenectomy in pediatric hematologic disease. Methods Sixty-eight patients who underwent splenectomy for various hematologic diseases were evaluated with regard to age, sex, blood count, and splenic mass. Patients who developed PVT were also reviewed for clinical features, treatment modalities, and outcome. Results Patients with PVT (n = 4, 5.88%) and without PVT (n = 64, 94.2%) had a mean age and female/male ratio of 13.2 years (range, 10-16 years) and 4:0, and 10.2 years (range, 1-16 years) and 29:35, respectively. Postoperative thrombocyte levels and splenic mass with and without PVT was 804 × 10 3 /mm 3 and 752.5 g, and 465.2 × 10 3 /mm 3 and 441g, respectively. Three patients with PVT presented with abdominal pain, fever, and vomiting. The diagnosis of PVT was made by Doppler ultrasonography in all patients including the asymptomatic case. Protein C, protein S, and antithrombin III levels were mostly decreased and/or normal and di-dimer levels were increased and/or normal after the development of PVT. Antiplatelet (acetylsalicylic acid) and antithrombotic therapy (low molecular weight heparin) were treatment agents. None of the patients needed surgery. During a mean follow-up period of 55.5 months, by Doppler ultrasonography, 1 patient was found to be free of thrombosis, whereas 1 had partial thrombosis. Two patients developed cavernomatous transformation leading to portal hypertension. Conclusions Portal vein thrombosis is a rare but significant complication of splenectomy done for hematologic diseases. According to our results, female gender and decreased levels of coagulation inhibitors seem to be risk factors in addition to previously mentioned thrombocytosis and greater splenic mass. Doppler ultrasonography may be performed in all patients after splenectomy to screen PVT. In the presence of well-known risk factors, prophylactic antiplatelet and antithrombotic therapy should be considered after splenectomy.

To analyze changes in the overall survival (OS) rate of children with Wilms tumor treated in a single institute over nearly 30 years. This study included 327 children with a newly diagnosed Wilms tumor. Their median age was 3 years, and the male:female ratio was 1.1. Survival rates were analyzed according to the stage of disease, histopathology, and different treatment regimens used between 1972 and 1999. At diagnosis, 51.1% of patients had advanced stage disease. Ten patients had anaplasia, and; 97% (317 patients) of the tumors had favorable histopathology. The 10-year OS rate was 60.6% for the entire group, but varied according to the years in which the patients were treated, the chemotherapy regimen, and stage of disease. Patients treated during the periods of 1972 to 1979, 1980 to 1989, and 1990 to 1999 had 10-year OS rates of 48.5%, 64.3%, and 72.8%, respectively. The 10-year OS rate in children treated with actinomycin only was 36.7% compared with 48% for children treated with the actinomycin-D+vincristine regimen with a 3-month interval, 67% for the actinomycin-D+vincristine regimen with a 1.5-month interval, 54.5% for the poor-risk regimen (actinomycin-D, vincristine, cyclophosphamide, and adriamycin), and 53.4% for the SIOP-9 protocol. Children with stage I to IV disease had 10-year OS rates of 75%, 77.1%, 54.4%, and 30.4%, respectively. The 10-year OS rates for children with stage III and IV disease increased from 46.4% and 13.4% for patients treated between 1972 to 1979 period to 75% and 54.5% for children treated during 1990 to 1999. The 10-year OS rate for children with Wilms tumor improved as treatment strategies evolved, illustrating that pediatric oncology in Turkey is developing parallel to the Western world.

Hepatic resection is the main treatment modality for hepatic tumors in childhood. Advances in diagnostic technique, preoperative preparation, surgical technique, and postoperative management increased the success rate. The aim of this study is to report our experience in hepatic lobectomy, which is relatively rare procedure in childhood. Medical records of 25 patients who underwent hepatic lobectomy between January 1977 and June 2002 were reviewed retrospectively. Age, gender, diagnosis, physical examination findings, results of preoperative laboratory investigations, radiological examination, resectability criteria, preoperative biopsies, chemotherapies, radiotherapies, postoperative pathological results, incisions, operation technique, intraoperative transfusions, drains used, antibiotic prophylaxes, and intraoperative and postoperative complications were evaluated for all patients. Out of 25 patients with hepatic tumor seven patients with hepatoblastoma and four patients with hepatocellular carcinoma were given 5.7 +/- 0.3 cycles of chemotherapy before the operation. Right lobectomy (n = 12), left lobectomy (n = 5), extended left lobectomy (n = 4), and extended right lobectomy (n = 3) and right lobectomy with enucleation of two masses from left lobe (n = 1) were performed. Intraoperative blood transfusion of 30.7+/-6.0 ml/kg body weight was necessary. Pathological examination of resected tumors revealed hepatoblastoma (n=11), mesenchymal hamartoma (n = 5), hepatocellular carcinoma (n = 4), hemangioendothelioma (n=1), malignant mesenchymal tumor (n = 1), hemangioma (n = 1), cyst adenoma (n = 1), and metastasis of cellular mesoblastic nephroma (n = 1). Patients were observed in the intensive care unit for 3.4 +/- 0.3 days. Postoperative complications were sepsis (n = 1), disseminated intravascular coagulation (n = 2), fever (n = 3), jaundice (n = 3), intraabdominal abscess (n = 3), ileus (n = 2), and subdiaphragmatic abscess with pleural effusion (n = 1). Hepatic lobectomy is a major operation, which is feasible yielding curative results in children. Safe hepatic resections with acceptable blood loss can be performed by a technique relying on good anatomic dissection and surgical control.

Chest wall tumors (CWT) are rarely seen in childhood and surgery constitutes a complementary part of the therapy. The early and late results of CWT resection and chest wall reconstruction were evaluated retrospectively. The children who underwent chest wall resection for CWT between January 1990 and November 2003 were evaluated retrospectively. Seventeen children (male/female = 12/5, mean age: 7.58 years) underwent chest wall resection for CWT. Fifteen patients underwent initial biopsy (tru-cut, n = 8 or open biopsy, n = 7) and two underwent initial resection. The diagnosis was malignant tumor in 12 (70%) and benign in 5 (30%). They were Ewing's sarcoma (ES) (n = 4), primitive neuroectodermal tumor (PNET) (n = 3), Askin's tumor (n = 1), rhabdomyosarcoma (RMS) (n = 2), neuroblastoma (n = 2), osteochondroma (n = 1), aneurysmal bone cyst (n = 2) and hamartoma (n = 2). Preoperative chemotherapy was given to most patients with malignant tumor. All patients had only local tumor at the time of resection. Thoracotomy was performed in all patients. All tumor tissues with the affected rib/ribs were resected en bloc with the adjacent tissues. The number of resected ribs was 1 (n = 6), 2 (n = 7) and 3 (n = 4). Chest wall defects were repaired primarily (n = 8) or with grafts (n = 9). Dura (n = 4), Neuro-patch (n = 3) and Goretex (n = 2) were used for closure. Wound infection and pleural fistula occurred in one patient. Patients with benign tumor were free of complaints or complications during follow up. All patients with malignant tumor received postoperative chemotherapy. Local recurrence did not occur in all patients. Five patients developed distant metastasis and two died. Scoliosis was encountered in one patient during follow-up. Since most of the CWT are malignant and not initially suitable for surgical excision, the management includes tissue diagnosis either by tru-cut or open biopsy. Determination of malignant condition should be followed by an intensive chemotherapy. Chest wall resection is planned to control local disease. Chest wall reconstruction may be needed for large defects following resection of CWT. Prosthetic materials can be used safely. Early complications of the surgery are limited. The patients should be closely followed up for late complications such as scoliosis, restrictive pulmonary disease and for the development of metastasis, which is a part of natural course of malignant CWT in children.

Background purpose Surgical treatment of childhood bronchiectasis has not been discussed extensively because of decline in prevalence and experience with this disease. It remains controversial as to which children would benefit from surgery and surgical points that may affect the outcome. Therefore, a retrospective series was prepared to evaluate the results of surgical treatment of bronchiectasis in children. Methods The records of 54 children who underwent surgery for bronchiectasis between 1991 and 2002 were analyzed retrospectively for age; sex; clinical features; radiologic examinations; details of surgery including type of resection, operative morbidity, and mortality; and outcome. Results Fifty-four patients underwent 58 pulmonary resections during the study period. The mean ages at diagnosis of bronchiectasis and at the time of surgery were 7.80 ± 3.70 years (range, 1 to 15 years) and 9.25 ± 3.92 years (range, 1.5 to 17 years), respectively, with a male to female ratio of 5:4. The causes of bronchiectasis were lung infection (n = 39), hereditary and inborn diseases (n = 14), and foreign body aspiration (n = 1). Chest X-rays, bronchography (n = 12) or chest computed tomography (n = 43), and ventilation-perfusion scintigraphy (n = 13) were used, and pulmonary function tests (n = 21) and bronchoscopy (n = 54) were performed. The types of resections were lobectomy (63%), pneumonectomy (18.5%), lobectomy with segmentectomy (11.1%), segmentectomy (3.7%), and bilobectomy (3.7%). Four patients required a second operation. Forty-one patients (76%) had complete resection, and 13 patients (24%) had incomplete resection. Intraoperative and postoperative complications were encountered in 4 (7.4%) and 4 patients (7.4%), respectively. The course after surgery was well in 23 (42.5%), improved in 23 (42.5%), and unchanged or worse in 5 patients (9.4%). The mortality rate was 5.6%. Conclusions The decision for bronchiectasis surgery should be made in cooperation with the chest diseases unit. Anatomic localization of the disease should be mapped clearly by radiologic and scintigraphic investigations. The morbidity and mortality rates of bronchiectasis surgery are within acceptable ranges. Most of the children benefit from surgery, especially when total excision is accomplished. Pneumonectomy is well tolerated in children without increase in morbidity and mortality. Therefore, pneumonectomy may be preferred instead of leaving residual disease when bronchiectasis is unilateral.

Background/Purpose: Ureteropelvic junction (UPJ) obstruction causes histologic alterations both in ipsilateral and contralateral kidney. Because these alterations directly affect the fate of renal damage, definition of these alterations is of utmost importance from the clinical point of view. Thus, an experimental study is designed to determine the alterations of renal histology in response to partial and complete UPJ obstruction. Methods: Fifteen adult female New Zealand rabbits were assigned randomly into 3 groups (each containing 5 rabbits) according to the degree of unilateral UPJ obstruction as group I, sham operation was performed and served as the control group; group II, partial UPJ obstruction was made; group III, complete UPJ obstruction was made. The animals in group I and II were killed after 3 weeks, and animals in group III were killed after 2 weeks. Tissue samples were prepared and processed according to routine light microscopic tissue processing. Results: UPJ obstruction led to glomerulosclerosis, dilatation of proximal and distal tubules of loops of Henle, and dilatation of collecting tubes consistent with necrotic and apoptotic changes in ipsilateral kidneys. Severity of these degenerative changes depended on degree of obstruction. UPJ obstruction also led to histologic alterations on the contralateral kidneys such as glomerular edema, congested blood vessels, dilated tubuli, and necrotic and apoptotic changes in epithelia, which were more prominent in group III than group II. Conclusions: It is well known that compensating changes including increased blood flow and parenchymal hypertrophy occurs in contralateral kidney as a response to unilateral UPJ obstruction. However histologic findings of this study confirmed progression of parenchymal damage and presence of apoptosis in contralateral kidney for the first time.

Objective: Computed tomography (CT) virtual bronchoscopy is a noninvasive technique that provides an internal view of trachea and major bronchi by three-dimensional reconstruction. The aim of this study was to investigate the usefulness of virtual bronchoscopy in the evaluation of suspected foreign body aspiration in children. Materials and methods: Twenty-three children (12 girls, 11 boys) with a mean age of 2.4 years (8 months–14 years) who were admitted to emergency room with a suspicion of foreign body aspiration were included in this study. Chest radiograms, spiral computed tomography scans and virtual bronchoscopy images were obtained. Then, rigid bronchoscopy was performed within 24 h. Results: CT virtual bronchoscopy and conventional bronchoscopy revealed the location of the foreign body in seven patients. It was in the right main bronchus in four patients, in the right lower lobe bronchus in one patient, and in the left main bronchus in two patients. There was no discordance between two modalities. CT examination revealed hyperaeration of the ipsilateral lung in four patients, hyperaeration of the ipsilateral lung and mediastinal shift in one patient and bronchiectatic changes in one patient. CT detected no additional finding in one patient with a foreign body in the right main bronchus. In 10 of 16 patients without foreign body, CT examination demonstrated atelectasis, infiltration, peribronchial thickening, and paratracheal lymphadenpoathy. Conclusion: Helical CT scanning with virtual bronchoscopy should be performed in only selected cases with suspected foreign body aspiration. When the chest radiograph is normal and the clinical diagnosis suggests aspirated foreign body, helical CT and virtual bronchoscopy can be considered in order to avoid needless rigid bronchoscopy.

The aim of this study was to present the entire spectrum of pediatric bronchoscopy performed for foreign body aspiration (FBA), with emphasis on accuracy of diagnostic tools, technical aspects, and predictors of complications.Records of patients who underwent bronchoscopy for evaluation of FBA in our unit from 1991 to 2000, inclusive, were reviewed retrospectively. Age, sex, history of FBA, symptoms, results of radiologic studies, bronchoscopy findings, complications, and outcome were recorded and statistically analyzed to find out the most accurate diagnostic tool.A total of 740 bronchoscopies were done in 663 children (402 boys, 261 girls) presenting with a mean age of 3.1 +/- 0.1 years. FBA was confirmed in 563 (85%) patients, whereas normal bronchoscopic findings, signs of pulmonary infection, and endobronchial mass were noted in 43 (6%), 54 (8%), and 3 (0.4%) patients, respectively. There was significant difference between patients with and without FBA with regard to presence of definite history (91% v 54%), normal physical examination findings (14% v 46%), and normal radiologic findings (13% v 31%). However, none of these parameters or their associations were found to be reliable to predict the presence of FBA or clinical complications. Presence of history was the most sensitive (91%), accurate (84%), and specific (46 %) diagnostic tool. Bronchoscopic removal of-foreign bodies was succeeded in 558 (99%) children. Worsening of respiratory tract infection (n = 13), cardiac arrest (n = 6), laryngeal edema (n = 5), pneumothorax (n = 5), pneumomediastinum (n = 2), tracheal laceration (n = 2), and bronchospasm (n = 2) were the life-threatening complications observed in 21 (4%) patients with FBA and 14 (14%) patients without FBA (P.05). There were 5 (0.8%) deaths. Of these, 2 patients presented with cardiopulmonary arrest immediately after FBA. Although foreign bodies were removed as quickly as possible, cardiac arrest was irreversible. The remaining 3 patients died of complications of bronchoscopy as irreversible cardiac arrest during bronchoscopy (n = 1) and worsening of respiratory tract infection after bronchoscopy (n = 2).The third year of the life carries the highest risk for FBA. There are no specific symptoms and signs to make a clear-cut differential diagnosis between FBA and respiratory tract infection. Bronchoscopy is invariably indicated on the basis of reliable history alone even when symptoms are minimal, and imaging studies are negative. Secondary bronchoscopy should be done in patients with persistent signs and symptoms to rule out overlooked organic foreign body particles or to remove persistent granulation tissue to avoid long-term complications necessitating lobectomy. The long duration of the procedure, presence of dense granulation tissue, and type of foreign body are important predictors of complications. Bronchoscopy should be regarded as an expert procedure and done with great care to avoid lethal complications. Differential diagnosis of respiratory tract infection by various diagnostic tools is of utmost important to avoid morbidity and mortality related to needless bronchoscopy.

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.

From 1972 to 2000, 123 patients with solid tumors whose complaints had started in the first 28 days of life were retrospectively evaluated. Fifty-five patients were diagnosed in the first 28 days and 68 patients were diagnosed after 28 days. In the former group, 85.5% of patients had symptoms in the first day of life. In the latter group, 77.9% had the onset of symptoms in the first day. Tumor subgroups in the neonatal period included teratoma (34), neuroblastoma (11), rhabdomyosarcoma (3), Wilms tumor (1), and retinoblastoma (3), and the others (3). Three patients had other, less common tumors. In the second group the numbers were the following: for teratoma (32), neuroblastoma (15), germ cell tumors other than teratomas (8), rhabdomyosarcomas (4), the other soft tissue sarcomas (3), Wilms tumor (1), retinoblastoma (1), and other, rare tumors (4). There were 22 malignant tumors in the first group, and 44 in the second group. Fourteen patients in the first group died in the early postoperative period or with progressive disease. Nineteen of 44 patients died in the second group. Overall survival rates were 24.9% and 51.6% in first and second groups, respectively (p=0.015). Event-free survival rates were 14.7% and 47.7% in these groups, respectively (p=0.0063). This is the first report comparing clinical features and prognosis of tumors diagnosed in the first 28 days of the life with those diagnosed after 28 days. The prognosis was worse in infants diagnosed in the first 28 days of life.

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.

Etat: La pyelonephrite xanthogranulomateuse (XGP) est une inflammation chronique inhabituelle du rein. La pathogenie, le diagnostic et la prise en charge de la XGP pediatrique est encore obscure et ceci est consequence du nombre limite de cas. Neanmoins, une etude clinique retrospective etait realisee pour presenter un tableau de differents aspects de la XGP pediatrique basee sur une experience de 30 ans representant l'une des plus importantes series traitees dans un seul centre. Methodes: Les dossiers des enfants qui ont ete traites pour XGP dans notre unite de 1970 a 1999 ont ete revus. Les informations collectees pour chaque patient incluaient l'âge, le sexe, l'histoire medicale, les caracteristiques cliniques, les moyens diagnostiques, le traitement, les decouvertes histopathologiques et le devenir. Resultat: 17 enfants avec un âge moyen de 6,6+/- 0,8 representant 15 garcons et 2 filles etaient traites pour XGP. Huit patients avaient une histoire urologique et 4 d'entre eux subirent une chirurgie pour des calculs urinaires ou pour une exstrophie vesicale. Habituellement, les symptomes presentes etaient douleurs abdominales, fievre, perte de poids et anorexie. Une masse palpable du flanc etait la constatation la plus frequente. Le rein gauche etait concerne pour 10 patients et le rein droit pour 7 patients. Les moyens diagnostiques etaient l'urographie intraveineuse, la cystographie retrograde, le scanner (CT) et l'echographie. Les calculs renaux et/ou les calcifications et un rein non fonctionnel ont ete les constatations les plus frequentes. En combinant toutes ces explorations, le diagnostic pre-operatoire etait XGP (n = 2), abces renal et/ou peri-renal (n = 5), abces du psoas (n=2), rein non fonctionnel due a une pyelonephrite chronique (n=4), tumeur de Wilms ou carcinome a cellule claire du rein (n=4). La XGP etait veritablement diagnostiquee chez 2 patients seulement sur les constatations du CT. Le drainage du rein ou de l'abces psoique etait realise chez 8 patients comme traitement initial (traitement chirurgical chez 6 et drainage per-cutane chez 2). Les gestes chirurgicaux etaient une nephrectomie (n = 13), une nephrectomie partielle (n=2), une nephrectomie avec section d'une fistule reno-colique (n = 1), et une biopsie renale avec nephrostomie (n = 1). Les complications per et post-operatoires etaient une perforation colique (n=3), une infection parietale (n = 3). Les complications etaient notees seulement chez les patients qui subirent une nephrectomie sans drainage initial. L'examen histopathologique a montre une XGP diffuse ou focale chez 14 et 3 patients respectivement. Conclusion: La XGP doit etre incluse dans le diagnostic differentiel de tous les enfants se presentant avec un abces peri-renal ou du psoas, une masse renale et/ou un rein non fonctionnel associe ou non a une lithiase. Un examen clinique precis et une forte suspicion est necessaire pour aboutir au diagnostic pre-operatoire correct et au traitement approprie. CT semble etre la methode la plus performante. Nous recommandons le drainage per-cutane des abces et une antibiotherapie avant nephrectomie pour eviter les complications. Une nephrectomie totale est le traitement le plus sur pour les lesions diffuses, alors que des biopsies extemporanees associees avec une nephrectomie partielle est obligatoire pour le traitement correct des formes focales.

Introduction: The structure called the residual gubernaculum (G) has been evaluated to define its origin. Materials and Methods: Girls operated for inguinal hernia (n = 7) and boys operated for undescended testis (n = 12), inguinal hernia (n = 7), hydrocele of the cord (n = 1), and ectopic (n = 1) and entrapped (n = 1) testes were evaluated. Attachments of the structures coursing distally from the sacs were identified, and they were removed en block. Three samples, first from the distal part of the sac with the beginning of the fibrous structure, second from the midportion, and third from the most distal part, were prepared and stained with hematoxylin-eosin, trichrome and elastic van Gieson. Histologic structures within the samples were determined and compared with Mann-Whitney U test. Results and Conclusions: Structures called the residual G differed from the walls of accompanying sacs by only the absence of mesothelial lining. Therefore, the structure called the residual G in boys with an undescended testis and the round ligament in girls actually represent the obliterated processus vaginalis.

In addition to an increase in contractility, contracted fibers and small and electron-dense mitochondria have suggested an increase of cytosolic Ca2+ within the cremaster muscles (CM) associated with undescended testis. Therefore, the Ca2+ content of CM associated with an undescended testis was determined and compared with the Ca2+ content associated with inguinal hernia and hydrocele and in internal oblique muscles. CM samples from boys with undescended testis (n = 9), inguinal hernia (n = 15), and hydrocele (n = 7) and from girls with inguinal hernia (n = 8) were obtained. The calcium contents of these samples and of samples from internal oblique muscle (n = 6) were determined by atomic absorption spectrophotometry. While the Ca2+ contents of CM from boys with undescended testis and from girls with inguinal hernia were similar (p > 0.05), the internal oblique muscle and the CM from boys with inguinal hernia and hydrocele contained more Ca2+ than CM from boys with undescended testis (p < 0.05). Despite evidence of an increase in cytosolic calcium, the decrease in total calcium content suggests a decrease in the adenylyl cyclase activity, thus inhibition of influx of Ca2+, but an increase in phospholipase C activity, and generation of more inositol 1,4,5-trisphosphate and mobilization of calcium from internal stores. Since the sympathetic system acts through adenylyl cyclase in striated muscles and is sexually dimorphic, similar Ca2+ contents as encountered in girls suggest an alteration in the balance of autonomic innervation against the sympathetic system in boys with undescended testis.

L'achalasie est une maladie rare de l'oesophage chez l'enfant. De 1971 a 1999, 20 enfants avec mega-oesophage ont ete traites dans notre hopital incluant 2 patients qui nous ont ete adresses apres l'oesophagomyotomie. Il s'agissait de 13 garcons et de 7 filles (âge moyen 8,2 annees; de 2 a 15 ans). Les symptomes revelateurs etaient des vomissements (n = 18), des dysphagies (n = 11), une perte de poids (n = 5), des infections respiratoires recurrentes (n = 3), de la toux (n = 2) et une respiration bruyante (n=1). Le transit baryte oesophagien permettait de faire un diagnostic chez tous les patients. L'oesophagoscopie etait utilisee chez certains patients (n = 10). 19 patients ont subi une operation de Heller-Zaiger (œsophagotomie de Heller modifiee) soit par voie transabdominale (n=16), soie par voie transthoracique (n = 3), avec (n = 6) ou sans procede anti-reflux. La periode postoperatoire etait sans particularite chez tous les patients. Le suivi etait de 2 mois a 16 ans. Un peristaltisme faible ou absent persistait chez tous les patients au premier transit oesophagien de controle. Un reflux gastro-oesophagien a ete retrouve chez un patient seulement. Une disparition complete des symptomes etait notee chez 14 patients. Une dysphagie moyenne a moderee etait rencontree chez 5 patients et tous etaient evalues par endoscopie et transit gastro-oesophagien. Les dysphagies disparaissaient spontanement chez un enfant et apres dilatation chez l'autre. Un enfant a eu une dysphagie moderee apres une courte periode d'observation. Une stenose oesophagienne a ete vue chez les deux autres et a ete traitee par une oesophagocardioplastie (intervention de Heyrowsky et Wendel). L'achalasie peut etre consideree comme un diagnostic chez tous les enfants presentant une dysphagie persistante, des infections respiratoires recidivantes et des vomissements, en incluant les enfants traites pour reflux gastro-oesophagien. Le traitement de l'achalasie est la myotomie chirurgicale. L'oesophagomyotomie modifiee de Heller est le procede de choix. Il peut etre realise par voie abdominale ou thoracique. Le besoin de realiser une valve anti-reflux dans le meme temps demeure controverse. Le probleme postoperatoire le plus frequent est la dysphagie persistante. Elle peut se limiter d'elle-meme dans quelques cas et disparaitre durant le suivi. Les stenoses persistantes, apres oesophagocardiomyotomie peuvent etre traitees par l'oesophagocardioplastie de Heyrowsky ou de Wendel.

Background/purpose Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm. Although it is commonly seen in children, the number of childhood cases in the current literature is limited. Furthermore, malignant degeneration or transformation to lymphoma in the recurrent or residual IMT have directed attention to this interesting entity. Herein, the authors present their experience with IMT with special emphasis on diagnosis and treatment. Methods All records of children treated with diagnosis of IMT between 1977 and 1999 inclusive were evaluated retrospectively. Results Seven children were treated for IMT with the mean age of 9.14 +/- 2 years (range, 6 to 12 years). Male to female ratio was 5:2. Respiratory symptoms and clubbing were present in a patient with pulmonary IMT (n = 1). Abdominal pain (n = 3), fever (n = 2), and weight loss (n = 4) were encountered in intraabdominal IMTs. The most frequent physical finding was palpable intraabdominal mass (n = 4). Plain films showed nonspecific findings such as radiodense area in the hemithorax (n = 1), displacement of bowel segments (n = 2), air-fluid levels (n = 1), and amorphous calcification (n = 4). Ultrasonography and CT showed calcified masses in 4 cases. Except the case with intrathoracic IMT, all the tumors were located in the abdomen at various sites such as cardioesophageal junction (n = 1), left hepatic lobe (n = 1), mesentery of the small bowel (n = 2), and antimesenteric wall of the descending colon (n = 1), gastrosplenic region and porta hepatis (n = 1). Tumor sizes ranged from 3 x 2 x 2 cm to 15 x 15 x 13 cm. The masses were excised totally in all but one case. Infiltrated organs (esophagogastric junction, a segment of jejunum, and spleen, stomach wall, and renal capsule) were resected in 3 cases. Total surgical excision of IMT was considered adequate for treatment in 6 cases. One patient with aggressive IMT required further treatments such as immunomodulation and chemotherapy and died of neutropenic sepsis. Conclusions IMT is a benign neoplasm rarely presented with malignant features such as local invasiveness, recurrence, distant metastasis, or malignant transformation. IMT can be suspected preoperatively through some hematologic abnormalities and radiologic findings, but precise diagnosis should be made on the basis of histologic findings. Complete surgical resection and close follow-up are all necessary for appropriate treatment to avoid recurrences as well as unnecessary and potentially harmful therapy. The optimal management of locally aggressive and recurrent forms should be decided individually for each patient. J Pediatr Surg 36:908-912.

The early detection and surgical repair of diaphragmatic injury is vital for saving the life of symptomatic children suffering from trauma. Furthermore, an accurate diagnosis may be difficult, particularly in right-sided diaphragmatic injuries. Fifteen children with diaphragmatic injury treated at our department between 1977 and 1998 were evaluated retrospectively. They included 9 boys and 6 girls, and consisted of 8 left- and 6 right-sided injuries, and 1 midline retrosternal injury, due to a blunt (n = 13) or penetrating (n = 2) trauma. The most frequent symptoms were dyspnea (86.6%), and abdominal pain and vomiting (13.4%). The diagnosis was confirmed preoperatively in 13 patients based on chest X-ray (n = 7), gastrointestinal series (n = 3), barium enema (n = 1), and computed tomography and/or ultrasonography findings (n = 2). Among these, a diagnostic delay occurred in 3 patients with right-sided injuries. A primary repair was performed through a laparotomy (n = 14) or thoracotomy (n = 1). Postoperative intussusception was the most frequent complication (n = 2). Diaphragmatic injury must be considered in any child who has sustained a thoracoabdominal trauma. Serial chest X-rays should be taken especially in right-sided injuries in which a considerable diagnostic delay may occur. Further radiological methods may be necessary to confirm the diagnosis. In addition, postoperative intussusception may be encountered following diaphragmatic repair.

Malignant ovarian tumors of childhood are relatively rare and thus, management is still unclear. We reviewed our experience with these tumors to evaluate their histopathologic characteristics, treatment, and outcome.From January 1975 to December 1997, 56 patients had their malignant ovarian tumors diagnosed, treated, and followed-up in our institution. All tumors were completely excised when possible; otherwise, biopsy was performed. Staging was made according to Federation Internationale de Gynecologie Oncologique classification. Chemotherapy was recommended for all patients. Twelve cases were treated with vincristine, actinomycin, cyclophosphamide (VAC) before 1986; 12 with cisplatin, vinblastine, and bleomycin (PVB) from 1986 to 1989; and 23 with the bleomycin, etoposide, and cisplatin (BEP) regimen from 1989 to present. The Kaplan-Meier survival method was used to calculate the survival. The log-rank test was used to compare groups with respect to survival.Age range was 0 to 16 years (median 11 yrs; average 9.8 yrs). Only two patients were younger than 1 year. The most common presenting symptom was abdominal pain, occurring in 27 patients (48.2%). Thirty-three patients (60%) had total one-sided salpingo-oophorectomy and three patients had bilateral salpingo-oophorectomy. Nineteen patients had stage I, 15 had stage II, 19 had stage III, and 3 had stage IV disease. Dysgerminoma was the most common type. Overall survival (OAS) and event-free survival were 68% (median follow-up time: 71 mos) and 57%, respectively, after 22 years. Histopathology was not correlated with survival. Two important predictors for survival are age (P0.0001) and treatment protocol (P = 0.013). The BEP protocol was superior to the other regimens. The OAS was 74.6% in BEP, 55% in PVB, and 63.6% in VAC regimens.Although age at diagnosis and treatment with BEP regimen have major roles in determining prognosis of the ovarian tumors in childhood, for patients with advanced ovarian germ cell tumors, intensification of chemotherapy or the development of new approaches is necessary.

Purpose: The caliber of processus vaginalis is accepted to define the clinical outcome to be an inguinal hernia or hydrocele not based on any evaluation. The caliber of sacs and length of inguinal canals of boys and girls were evaluated to define the relation of sex, age, and the diagnosis with caliber of the sac and the length of inguinal canal. Methods: A total of 217 inguinal canals in 24 girls and 112 boys with inguinal hernia, 30 boys with hydrocele or hydrocele of the cord, and 31 boys with undescended testis have been evaluated. Twenty patients had bilateral involvement. The length of inguinal canal, and the circumference of the sac were measured. A formula was developed to predict the length of inguinal canal according to the age and sex. The circumferences of the sacs, length of inguinal canals, and the ratios of the circumference to the length were compared according to the clinical pictures. Results: The regression model of the relationship between the age and the length of the inguinal canal is an equation of third degree (inguinal canal in millimeters) = 0.0000119 × age 3 (months) − 0.00292 × age 2 (months) + 0.3168 × age (months) + 19.979 (r 2 = 0.47). Inguinal canal is longer in boys (25.133 and 27.996 mm; P =.018), and length does not differ among diagnoses but differs according to age showing a linear growth after 24 months. Although the circumference as a sole parameter could classify only 55.3% of boys correctly, the ratio of length of inguinal canal to circumference of the sac has been the significant parameter in classifying boys into 1 of 3 groups including inguinal hernia, undescended testis, and hydrocele with a 70.2% success rate. Conclusions: Inguinal canal that shows a linear growth after 24 months of age is longer in boys. Caliber is not the unique factor that determines the clinical outcome. Although the ratio of length of inguinal canal to the circumference of the sac defines the clinical picture best, even this parameter cannot classify the cases correctly. Therefore, some factors in addition to the caliber and length of inguinal canal might have roles in determining the clinical outcome. J Pediatr Surg 35:1165-1169. Copyright © 2000 by W.B. Saunders Company.

The intraabdominal pressure (IAP) of children presenting with acute, perforated, or suspected appendicitis were determined and compared to define if the IAP has any diagnostic value or helps to predict a complicated course.Eighty-four patients with a initial diagnosis of appendicitis were evaluated. In addition to preoperative measurements, IAP of each patient was determined repeatedly on the postoperative first, second, and third days. The patients were grouped according to the final diagnoses as acute, perforated, or suspected appendicitis or negative exploration. The preoperative and postoperative IAP of the patients were compared among the groups. Postoperative complications were recorded, and IAP of those patients were additionally compared with the others in the same group.Whereas a normal appendix was found in 4 of the operated patients, 27 and 38 patients had acute and perforated appendicitis, respectively. The mean preoperative values of IAP for acute, perforated, or suspected appendicitis and negative exploration were 6.2 +/- 0.4, 9 +/- 0.3, 0.3 +/- 0.4, and 3 +/- 0.4 cm H2O, respectively (P.001). Postoperative first day and second day values of the IAP for acute appendicitis, perforated appendicitis, and negative laparotomy groups were 2 +/- 0.2 and 0.6 +/- 0.1,3 +/- 0.1 and 1.5 +/- 0.1,0.5 +/- 0.6 and -0.2 +/- 0.6 cm H2O, respectively. The difference between acute and perforated appendicitis groups was significant (P.05). Wound infection was encountered in 7 among 38 patients with perforated appendicitis. The preoperative and first postoperative day IAP values of patients with perforated appendicitis who experienced a wound infection and who were without a wound infection have been 11.8 +/- 0.4 and 4.8 +/- 0.2, and 8.4 +/- 0.2 and 3.1 +/- 0.3 cm H2O (P.001). Discriminant analysis has shown that 93.3%, 70.4%, and 73.3% of patients with suspected, acute, and perforated appendicitis have been within the expected groups. IAP less than 1.39 cm H2O has excluded appendicitis with a 95% confidence interval. Although the interval has been between 5.40 and 7.04 cm H2O for acute appendicitis, it has varied between 8.46 and 9.70 cm H2O for perforated appendicitis.Although the IAP does not increase in conditions mimicking appendicitis, it increases among children with appendicitis. A further increase is encountered among children with perforated appendicitis. Complicated course is encountered among children with highest IAP values. Therefore, IAP may be used both as a diagnostic parameter and a predictor of a complicated course associated with appendicitis in children.

Background/Purpose: The mechanism of testicular descent remains controversial. The processus vaginalis (PV) contains smooth muscle and should have contractile activity that may contribute to descent. This study was designed to evaluate the smooth muscle of PVs associated with incomplete obliteration for spontaneous activities and responses to various stimuli, to determine if differences exist according to sex, diagnostic source, or location of the testis. Materials: Peritoneal samples (n = 4); sacs from girls (n = 8) and boys with inguinal hernia (n = 12); and sacs from boys with hydrocele (n = 3), hydrocele of the cord (n = 2), or undescended testis (n = 7) were used for the current study. Tissues were attached to the isometric force displacement transducer in an organ bath containing mammalian Ringer's solution at 37°C. Spontaneous mechanical activity and contractile responses of tissues to the electrical field stimulation, phenylephrine, carbachol, and serotonin were recorded. The values obtained from boys and girls with inguinal hernia and from boys with either undescended or descended testis were compared through Fisher's Exact test. Results: There were no statistically significant differences in patient age between groups. Among the parameters studied, only the carbachol response of the sacs associated with undescended testis showed a significant difference compared with the others ( P = .001). None of the sacs associated with undescended testis responded to carbachol, whereas all of the sacs from boys and girls with inguinal hernia responded to carbachol. Conclusions: Lack of carbachol response suggests the absence of cholinergic receptors within the sacs associated with undescended testis. The lack of cholinergic receptors may play a role in the failure of the process of testicular descent by hindering either PV elongation into the scrotum or a possible propulsive activity of the PV on the testis.

Background/Purpose: Entamoeba hystolytica (EH) is an enteric protozoan that may cause serious complications that require operative management in children. A retrospective clinical trial was performed to find out the incidence and outcome of complicated intestinal amebiasis (IA) by means of surgically resected specimens in children. Methods: The histopathologic evaluation of 554 intestinal specimens (including appendix and polyp) of 482 patients between 1980 and 1997, inclusive, were reviewed. Intestinal biopsy specimens taken from successfully medically treated patients with uncomplicated IA were excluded. Results: Complicated IA was noted in 18 children (3.7%) consisting of 7 girls and 11 boys with a mean age of 9.2 ± 2.3 years. EH was present in the (1) appendices of 4 patients with perforated appendicitis and 3 patients with normal appendix who underwent negative exploration; (2) juvenile polyps of 4 patients presenting with hematochesia; (3) colonic wall of 1 patient presenting with intussusception and treated by resection and anastomosis; (4) cecum of 1 patient presenting with right lower quadrant mass and underwent exploration with the presumptive diagnosis of lymphoma; (5) colonic wall of 5 patients who underwent ileostomy followed by subtotal colectomy and endorectal pull-through with the presumptive diagnosis of chronic inflammatory bowel disease (n = 3), toxic megacolon + peritonitis (n = 1), and total colonic polyposis (n = 1). All above-mentioned patients are alive and free of symptoms. Conclusions: The diagnosis of IA should be considered in a very wide spectrum of clinical appearances. IA may be associated with colonic polyps and perforated appendicitis, may act as a leading point for intussusception, and may mimic the clinical picture of appendicitis and lymphoma resulting in negative explorations. The diagnosis and treatment of complicated IA in patients who received a misdiagnosis of inflammatory bowel disease is a great challenge and requires major and emergency surgery.

Purpose: An experimental study was performed to evaluate the effect of fetal distress on in utero defecation and clearance of amniotic fluid (AF). Methods: Sixteen pregnant New Zealand white rabbits underwent laparotomy at 25 days' gestation (full term, 31 to 32 days) as group A (n = 8) and B (n = 8). Uteroplacental ischemia was achieved by constriction of the aorta below the renal arteries to cause fetal distress in group B, whereas sham operation was done in group A. In both groups, 0.1 mL of technetium-99m ( 99m Tc)-HIDA containing 1 mCi of radioactivity was injected into the gluteus muscle of each fetus, which had been exposed through the uterus. Beginning 2 hours after injection, a live fetus was killed every 2 hours for 48 hours in both groups. Tissue samples from the reference organs (lung, heart, stomach, kidney, bladder) and liver, meconium in proximal, mid and distal bowels, AF, and maternal blood were taken. The radioactivity of each sample was determined by a gamma counter and the percentage injected dose (uptake) per gram of tissue (%ID/g) was calculated. The total uptake and mean transit time (MTT) showing intestinal transport were calculated using the linear trapezoidal approximation and extrapolation. The peak concentration (C max , %ID/g) and time corresponding to the peak (t max , h) were obtained. Results: (1) Significant difference was noted between the groups with regard to uteroplacental perfusion pressure and blood pH (51.0 ± 2.6 mm Hg; pH, 6.9 ± 0.1 in group B; 80.1 ± 2.7 mm Hg, pH, 7.3 ± 0.1 in group A; P 99m Tc-HIDA was predominantly trapped by the liver and excreted into the gastrointestinal tract and AF in both groups. (3) In liver and bowel, shape of the profile was bimodal because of fetal swallowing and similar in both groups, t max was the same in both groups, C max was lower in group B than in group A, the total uptake was smaller in group B than in group A, and intestinal transport time was similar (44.2 hours in group A and 43.0 hours in group B). In amniotic fluid, shape of the profile was sigmoidal in group B and reached a C max value of 11.6% ID/g, whereas unimodal profiles were observed in group A with a C max value of 12.6% ID/g; radioactivity was eliminated from the AF with a rate constant of 0.48% ID/g h in group A (AUC, 273% ID/g h); whereas accumulation of radioactivity was noted in group B (AUC, 308% ID/g h). (5) In maternal blood, shape of the profile was sigmoidal in group A with a C max value of 2.9% ID/g and unimodal in group B (C max , 1.6% ID/g), accumulation of radioactivity was noted in group A (AUC, 93% ID/g h), whereas a rapid decline of radioactivity (k, 0.06% ID/g h) was noted in group B (AUC, 47% ID/g h). Conclusions: Fetal distress did not affect the intestinal transport dynamics and in utero defecation but impaired the clearance of AF and the passage into the maternal circulation, which was shown by the accumulation of radioactivity in AF only in group B and in maternal blood only in group A without any elimination rate. This finding suggests that meconium-stained AF is not related to meconium passage after fetal distress; rather, it reflects impaired clearance of AF, which already has containing meconium caused by physiological in utero defecation.

A 14-year-old girl presented with malignant hypertension. Physical examination and abdominal computerized tomography revealed a paraaortic mass. Urinary catecholamines, serum renin, and aldesterone levels were high. The mass was totally excised and a paraganglioma of the organ of Zuckerkandl was confirmed. The hypertension resolved after surgery.

Between 1976-1993, 302 patients were diagnosed to have Hirschsprung's disease (HD) in Hacettepe University Children's Hospital. All patients have been searched for the occurrence of associated anomalies and other parameters including 1) sex, 2) gestational age, 3) birth weight, 4) length of aganglionic segment, 5) familial occurrence, 6) consanguinity. Forty-nine of 302 patients were proved to have 83 associated congenital anomalies together with HD. Distribution of anomalies was as follows; central nervous system and special senses 28.92%, genitourinary system 26.51%, skeletal 24.09%, gastrointestinal 12.05%, cardiovascular 8.43%. In eleven patients more than one anomaly involving different systems were detected; only one of these patients was female and six of them have shown short segment disease. Down's syndrome was present in 5 patients. Female: male ratio was 1:4.2 for the whole series and 1:6 for the patients with Hirschsprung associated congenital anomalies (HACA). In the HACA group the percentage increased in favor of long-segment and extensive aganglionosis without statistical significance (p>0.05). The majority of patients were term babies and their birth weights were between 2500g < 3500g. Consanguinity was present in 36 families. No affected parents were detected but two sisters and four brothers were proved to have HD. Three pairs of twins, one of which was monozygotic, and all showing discordance were detected. There was no difference between HACA group and HD without HACA in terms of sex, birth weight, length of aganglionic segment and consanguinity. Although HD seemed to be genetically heterogeneous HACA did not have a genetically different origin from that of HD.

Background: H-type tracheoesophageal fistula (TEF) was diagnosed in 12 patients during the 25 years from 1971 to 1996. Although all patients were symptomatic since birth, in 58% of the cases, there was a diagnostic delay ranging from 26 days to 4 years. Methods: Esophagography and/or cineesophagography, or bronchoscopy and simultaneous methylene blue administration confirmed the diagnosis of H-type TEF. Although cineesophagography is highly effective in demonstrating H-type TEF today, bronchoscopy must be used in every patient suspected of having fistula, especially when the radiological methods fail. Bronchoscopy is helpful in diagnosis, in evaluation of associated respiratory tract anomalies, and in treatment. Results: Two newborn patients could not have been operated on because of pulmonary insufficiency resulting from delayed recognition of fistula. Ten patients underwent fistula repair, nine through cervical approach, and one through thoracotomy, with seven survivors. Conclusions: The results suggest that early diagnosis is essential in the newborn period. Oversight in identification of H-type TEF, unsatisfactory radiological method, masquerading symptoms related to associated anomalies, and minute symptoms in some patients may cause delay in diagnosis. Radiological and endoscopic procedures are complementary in both diagnosis and treatment of H-type TEF.

Pulmonary lymphangioleiomyomatosis (PLAM) is a rare, diffuse progressive interstitial lung disease that affects females of childbearing age and is characterized by diffuse proliferation of abnormal smooth muscle fibers predominantly developing in the lung and leading to cystic destruction. A prepubertal 13-year-old girl with PLAM associated with renal and hepatic angiomyolipomas who was treated by nephroureterectomy and thoracoscopic pleurodesis is presented. To the best of our knowledge, this is the first reported case of PLAM associated with renal and hepatic angiomyolipomas at the prepubertal age. After evaluating the clinicopathologic features of this rare entity, the authors conclude that PLAM should be considered in the differential diagnosis of cystic pulmonary pathologies in children, particularly teenagers. The most important clinical aid is to bear this rare entity in mind when a child presents with renal and/or hepatic angiomyolipomas. Thoracoscopic pleurodesis is the most effective treatment modality for recurrent pneumothoraces.

A 1-year-old girl with Waardenburg syndrome type I presented with double collecting system of left kidney accompanied by nonobstructive hydronephrosis of lower pole and by ureteropelvic junction obstruction of right kidney. Renal involvement in Waardenburg syndrome was reported once in a 4-month-old boy with unilateral duplication of the renal collecting system and in a 16-day-old girl who had right multicystic dysplastic kidney and hydronephrosis in the left kidney. The third case of renal involvement in Waardenburg syndrome is presented here, with special emphasis on early diagnosis and management of renal anomaly. The authors conclude that urinary system anomalies should also be considered in the wide spectrum of Waardenburg syndrome clinical features to avoid life-threatening complications.

229 patients atteints de maladie de Hirschsprung qui ont eu une operation definitive entre 1976 et 1993 ont ete etudies pour preciser la survenue de complications inhabituelles dans 1'Hopital d'Enfants de l'Universite d'Hacettepe. 213 de ces patients ont ete operes dans notre Hopital et 16 nous avaient ete adresses apres l'operation definitive, la complication la moins exceptionnelle, dans cette serie, etait la fistule, en particulier recto-urinaire, recto-vaginale, ileo-rectale et peri-anale. Il patients operes par la technique de Swenson ont presente une fistule recto-uretrale qui a necessite une reintervention de Swenson apres une tentative de reparation directe non suivie de succes. Dans cette serie, nous avons detecte 2 patients avec fistule ileo-rectale, l'un entre eux avait egalement une fistule peri-anale. 2 patients ont presente une enterocolite rebelle a tout traitement; il s'agissait chez ces 2 malades d'une fistule ileo-rectale qui a pu etre reparee avec succes. Nous avons rencontre 2 fistules recto-vaginales chez des patients qui nous ont ete adresses apres operation definitive, ces deux patients ont ete reoperes. Un patient a ete adresse apres une operation de Swenson avec une fistule peri-anale et a subi une fistulotomie. Le traitement des fistules recto-urinaires et recto-vaginales doit etre une reintervention complete sinon il aboutit toujours a un echec.

It is well known that Helicobacter pylori infection is extremely common throughout the world, but most infected persons do not develop ulceration and remain asymptomatic. This study was undertaken in order to determine the prevalence of H. pylori infection, the age distribution, and the factors that may affect the frequency of H. pylori infection. We evaluated the presence of antibody against H. pylori in children and adolescents, we also assessed the efficacy of non-invasive and invasive methods for detection of H. pylori infection in children who had undergone upper gastrointestinal endoscopy. We evaluated 43 males and 18 females whose ages ranged from 1 to 17 years (mean age: 9.4 +/- 4.2). 29 of all cases were symptomatic and 32 were asymptomatic. H. pylori infection was present in 49% of the studied cases. The data were analysed by Fisher's exact chi-square and Mantel-Haenszel tests. It was found that H. pylori infection graphically increased with age in both groups from 25% at ages 3-5 to 80% at ages 16-20. But no association was determined statistically between age and H. pylori positivity (p0.05). H. pylori infection was determined in 14 (48%) and 16 (50%) cases in symptomatic and asymptomatic groups respectively. There was no significant difference between H. pylori and either group (p0.05). According to the endoscopy findings, the symptomatic group was divided into two subgroups: children with peptic ulcer and children with non-ulcer dyspepsia (NUD). An attempt was made to compare ulcer, non ulcer dyspepsia and asymptomatic cases with H. pylori positivity, but no significant relation could be established between the groups (p0.05). 14 (23%) of the cases had previous gastric and/or intestinal complaints in their parents (positive family history). There was a significant, statistically positive relation between family history and symptoms (p0.01). Nevertheless, no association was found between H. pylori infection and family history (p0.05). There were no significant differences in H. pylori infection related to sex, type of housing, location of housing, socio-economic status (SES) or source of water supply. It is concluded that H. pylori infection has a high prevalence in our country. Although endoscopic evaluation is an invasive method, under our conditions, histopathological examination with 97% is shown to be much more sensitive in determination of H. pylori infection. Since no community difference was determined in the prevalence of H. pylori infection, all children should be considered to be under the threat of the same risk.

Quoique la chirurgie thyroidienne soit principalement basee sur le risque de malignite, la determination des cas de malignite thyroidienne chez les patients porteurs d'une thyroide volumineuse, est un sujet de discussion chez les enfant. Une etude clinique retrospective a ete entreprise pour definir quels types d'augmentation de volume de la thyroide ont une potentialite clinique ou histologique de malignite. Au total, 137 patients qui ont subi une chirurgie thyroidienne pour tumeur sur une periode 15 ans ont ete etudies. La serie est composee de 90 cas (65,7 %) de goitre uninodulaire, 40 cas (29,2%) de goitre multinodulaire et 7 cas (5,1 %) de goitre diffus. Les examens histopathologiques ont trouve 9 patients (6,6 %) porteurs d'un carcinome thyroidien differencie. Tous les autres patients avaient des tumeurs benignes. L'analyse des resultats a montre qu'il n'y avait pas de possibilite de prediction ideale d'une malignite thyroidienne, pas plus sur le plan clinique ou les caracteristiques de laboratoire que sur les notions d'âge, de sexe, les symptomes evocateurs, une irradiation anterieure, le type de nodule, la localisation et le siege des nodules, les adenopathies lymphatiques, les tests de fonction thyroidienne, l'echographie et le scanner thyroidiens. Toutes ces caracteristiques etudiees n'ont pas de signification importante pour une valeur predictive positive ou negative. Seulement deux parametres montrent une specificite elevee au dela de 50 % pour le diagnostic d'une tumeur vraisemblablement maligne : une taille d'au moins 2,5 cm associee a des adenopathies cervicales. En consequence, de nouvelles regles plus valables doivent etre recherchees pour l'evaluation chirurgicale thyroidienne chez l'enfant ; la biopsie par aspiration a l'aiguille fine qui est utilisee couramment chez l'adulte peut etre une approche valable en depit de quelques notions restrictives chez l'enfant.

Posttraumatic prostatomembranous urethral strictures (PUS) in children have special features that warrant consideration with respect to management. Transpubic urethroplasty (TPUP) was used to treat 10 children who presented with posttraumatic PUS. All patients previously had unsuccessful repair attempts with other techniques. Urethral stricture was noted in three patients who were cured within a few months by urethral dilatations. Appropriate antibiotic therapy was begun for prolonged urinary infection in four patients. Complete urinary continence was achieved in seven patients (70%), and three (30%) are still incontinent. In the latter, the bladder neck and posterior urethra were reported to have been damaged seriously, owing to extensive dissections at the time of initial treatment. Severe retropubic tissue loss and fibrosis were noted in these patients. Thus, the authors emphasize that avoiding further injury to any of the continence mechanisms that have survived the injury is as important as performing a patent urethral anastomosis. There were no significant complications or morbidity associated with TPUP. TPUP provided excellent exposure and visualization of the involved anatomy and facilitated accurate suture placement and tissue realignment in all patients.

Pseudocyst of the pancreas is an uncommon disorder in children. During the period of 1977 to 1990, the authors reviewed the records of 10 consecutive patients with pancreatic pseudocyst (PP) who were surgically treated in our institution. In 8 patients, the surgical procedure that was used was transgastric cystogastrostomy, and in 1 patient it was total excision of the pseudocyst. The remaining 1 patient underwent external drainage. After an average follow-up of 8.5 years, none of the patients had any complaint related to previous operation except the 1 patient who was treated with external drainage. Biochemical tests, abdominal ultrasonographies, and barium meals were also normal in 9 patients. The analysis of the results indicates that despite new percutaneous therapeutic modalities, it is still an internal drainage through transgastric cystogastrostomy that should be favored in treatment of PP in children.

Gastrointestinal stromal tumor is a rare pathology in childhood. It may occur anywhere along the alimentary tract and represents with a wide spectrum of signs and symptoms according to location. An infant with malignant colonic stromal tumor that has presented with a huge abdominal mass and lower gastrointestinal bleeding is reported to discuss the difficulties in diagnosis and differential diagnosis of this unusual tumor.

An experimental study was performed to investigate gastrointestinal motility and meconium passage, with simultaneous blood gas measurements, in the fetuses of eight pregnant goats at 110 to 114 days' gestation (full term, 147 to 155 days). With the goats under halothane anesthesia, a nasogastric tube and a heparinized central venous catheter were inserted into the fetuses. 24 hours after surgery, 10 mL of gastric juice from the fetus was replaced with a nonhydrosoluble contrast medium, and serial roentgenograms and blood samples (for pH, PO2, and PCO2 measurement) were taken every 4 hours. All fetuses began to pass the contrast medium into the amniotic cavity within 16 to 22 hours, and central venous blood gas values were normal. The results of this study suggest that the fetus, which urinates routinely, also defecates routinely into the amniotic cavity, even in the absence of distress. The increased incidence of meconium staining in fetal distress conditions may be indicative of impaired clearance of amniotic fluid.

All the complications requiring laparotomy among 680 children who were diagnosed as having typhoid fever between 1981 and 1990, at Hacettepe University Children's Hospital, were retrospectively analyzed. Four patients developed intestinal perforation, two gastrointestinal bleeding, and two splenic abscess, with frequencies of 0.58%, 0.29%, and 0.29%, respectively. The overall frequency was 1.17%. While the risk was 1.58% among males, it was 0.42% among females with no statistically significant difference (P > .05). The risk was higher among patients above 6 years of age (P < .05). While perforation and hemorrhagic complications developed within 10 days of initial symptoms and splenic abscess required 10 and 30 days for development, there were no clinical signs and laboratory findings predicting the occurrence of complications in the course of typhoid fever. Two patients, one with intestinal perforation and the other with gastrointestinal hemorrhage, died within 2 days following the septic postoperative courses. On the other hand, 12 patients, among those without complications requiring laparotomy, died of unremitting septic course of the disease. The mortality rate rose from 1.78% to 25% when complications occurred. The findings in this series demonstrate that typhoid fever presents a challenge to the pediatric surgeons not only because of the complications requiring laparotomy with high mortality rates, but also the absence of criteria predicting the occurrence of complications in the course of typhoid fever.

In childhood, benign tumors of the cervix and corpus uteri are very rare. Clinical and pathological findings of a benign multilocular cystic polyp of the cervix uteri in a 5-year-old girl are presented and the origin of the polyp is discussed.

Thoracic neuroblastic tumors (NBTs) are reported to have better prognosis. We aimed to review clinical characteristics, treatment results, and outcome of our patients with thoracic NBT.Files of 87 children treated at our hospital between 1973 and 2007 with the diagnoses of thoracic NBT were reviewed for clinical and pathological characteristics. Treatment results and outcomes of these cases were examined.All but one tumors were located in posterior mediastinum, one in the posterior chest wall. Median age of all was 2.1 years (range, 0.03-14; F/M: 1.42). Fifteen cases had ganglioneuromas (GN), 26 ganglioneuroblastomas (GNBL), and 46 neuroblastomas (NBLs). Stages were: I, 20.5%; II, 22.1%; III, 38.2%; IV, 14.7%; IVS, 4.5%. Stages III and IV were more common in cases over 1 year of age. In 20 patients diagnoses were incidental. Twenty-two of 87 (25.3%) had symptomatic spinal cord compression and 15 (17.3%) had Horner syndrome. Ten-year overall and event-free survival rates were 71.2% and 67.4%, respectively. Survival rates did not differ depending on the age being younger or older than 1 year. Ten-year survival rates were 88.8% in stages I, II, IVS; 65.3% in stage III and 27.8% in stage IV (P = 0.0002).Thoracic NBLs had a favorable prognosis. This might be a result of earlier diagnosis and some distinct biological characteristics. Favorable prognosis would suggest less aggressive treatment for such patients. Further studies on the biological characteristics of NBLs in the thoracic site and their association with outcome should be done.

From 1972 through 1991, 404 patients with renal tumor (Wilms' tumor and clear cell sarcoma) were seen at the Pediatric Oncology Unit of Hacettepe Children's Hospital. The genital abnormalities and renal diseases in these patients were evaluated retrospectively. Eight patients with renal tumor had genital abnormality and/or renal disease of various types and degrees. One of these patients had a clear cell sarcoma while all the others had Wilms' tumor. Two patients had all components of the Drash syndrome. Two patients with Wilms' tumor had genital abnormality and mild proteinuria. Another two patients had only Wilms' tumor and renal disease. Two patients had only renal tumor and genital abnormality of whom one was the patient with clear cell sarcoma.

An 11-month-old girl with a prolonged history of bloody, mucoid diarrhea is presented. Although the initial diagnosis given by the rectosigmoid biopsy obtained during laparotomy was neuronal intestinal dysplasia, accompanying findings including mixed inflammatory cell infiltration of the mucosa and submucosa with mucosal ulcerations suggested nonspecific colitis. The subsequent biopsy specimen that was obtained after performing colostomy and treating with broad-spectrum antibiotics and rectal irrigations showed improvement in the structure of ganglion cells and submucous and myenteric plexuses. Although the mucosal ulcerations and inflammatory reaction improved, the colonic stricture persisted, so the Duhamel procedure was performed, and the patient had an uneventful outcome. It is claimed that inflammatory disease of the rectosigmoid colon of unknown etiology and neuronal intestinal dysplasia have occurred together in the current case or that one disease might cause the other in time.

The records of 230 adhesive small bowel obstruction (ASBO) episodes in 181 patients have been reviewed to observe the place of conservative treatment and to establish criteria to predict the success of conservative trial. Immediate operation has been reserved for 81 episodes that have presented with fever and leucocytosis and/or localized abdominal tenderness, or complete obstruction. The remaining 149 episodes have initially undergone conservative trial. Although 110 episodes (73.8%) have been cured with conservative trial, 39 (26.2%) subsequently necessitated surgical intervention. No adverse occurrences have been observed during or after delayed operations. There was no strangulated bowel nor mortality both in delayed operation and conservatively treated groups. Recurrence has occurred with rate of 18.75% and 36.47% after surgery and conservative treatment, respectively, being significantly different (P less than .01), but the treatment method of the previous episode has been without influence on the method used in the recurrent obstruction (P less than .05). Among the assumed predictive criteria, age at recent laparotomy (P less than .02), time elapsed between recent laparotomy and obstructive episode (P less than .01), the primary condition necessitating laparotomy (P less than .01), the incision of previous laparotomy (P less than .05), and duration of conservative trial (P less than .01) correlated significantly with the success of conservative trial. The number of previous laparotomies and obstructive episodes (P less than .05) have not showed correlation. By the conservative approach used in selected patients with ASBO, 40% overall have been spared operation, without any adverse occurrences. Using the proposed criteria, the success rate of conservative treatment can be predicted.

In this research, we aimed to evaluate the accordance of radiologic study results with each other and with surgical findings with regard to presence and/or absence of intussusception. One hundred and seventy-nine patients treated for intussusception between 1993 and 2003 inclusive were retrospectively reviewed to compare results of initial ultrasonography, colonography, followup ultrasonography after conservative management (reduction with barium enema and/or air insufflation) and surgical findings to determine their accordance within each other with regard to diagnosis of intussusception. Results of initial ultrasonography were not in accordance with results of colonography. There was accordance between conservative management and follow-up ultrasonography results. Conservative management results were not in accordance with surgical findings. There was no accordance between followup ultrasonography results and surgical findings. Discordance of radiologic examination results with each other and with surgical findings indicates that intussusception is still a clinical diagnosis and clinical parameters deserve more importance in surgical decision-making. Radiologic examinations should be considered as complementary studies, not as definitive discriminators of childhood intussusception to achieve appropriate diagnosis and treatment.

Gastric volvulus is a rare entity in children. During the last ten-year period of 1977 to 1987, 21 children were operated upon with the diagnosis of chronic gastric volvulus at The Department of Paediatric Surgery, Hacettepe University School of Medicine. This study includes retrospective analysis of these cases. The patients were investigated with respect to associated anomalies, radiological diagnosis, surgical treatment and prognosis. Results are presented and discussed.

Purpose: A retrospective clinical study was performed to find out whether serum biochemistry alterations may serve to differentiate the traumatic bladder perforation to be either intraperitoneal or extraperitoneal. Methods: Thirty-two children treated for traumatic bladder perforation between 1970 and 1997, inclusive, formed the study group. Patients were divided into two groups: intraperitoneal bladder perforation (IBP) and extraperitoneal bladder perforation (EBP) groups. The groups were compared with regard to age, sex, mechanism of injury, and hemodynamic parameters (blood pressure, hematocrit) at presentation (each group was further subdivided into two subgroups according to the duration between trauma and presentation as early (duration 24 hours) presentation groups). The subgroups were compared with each other with respect to serum concentrations of urea, creatinine, sodium, potassium, and chloride. Results: There were 21 boys and 11 girls with a mean age of 8.7 ± 4.9 years. Mechanisms of injury were motor vehicle accidents (75%), falls (19%), and gunshot wounds (6%). There was no significant difference between the IBP and EBP groups with regard to age, sex, mechanism of injury and hemodynamic parameters at presentation. The biochemical parameters were found to be within normal range in the EBP group, whereas significantly higher levels of creatinine, potassium, and lower level of sodium were noted in IBP group regardless of presentation time. Significantly higher level of serum urea was recorded in a late presentation group of IBP patients. Conclusions: The authors emphasize that patients presenting with IBP are more likely to present with significantly higher levels of creatinine, potassium, and lower level of sodium compared with the patients with EBP regardless of presentation time. Increased level of serum urea concentration is a characteristic of IBP patients with late presentation. Biochemical alterations can be used to differentiate traumatic IBP and EBP in children with subtle physical examination and radiological findings.

We aimed in this study to evaluate the clinical and radiological features of the late recurrence of Wilms' tumor in children. Among 553 children diagnosed with Wilms' tumor between 1972 and 2004, four cases were determined to be late recurrences. Clinical, histopathological parameters, treatment details, and outcomes of the patients were evaluated retrospectively. The ages of the patients at the time of diagnosis were 2, 5, 5, and 9 years and the male/female ratio was 1/3. Two patients had stage II disease and two had stage IV characteristics. Histopathological examination showed favorable histology in all of the patients. Initial treatment was surgery and chemotherapy, which included vincristine and actinomycin-D. Abdominal radiotherapy was performed in two patients. Recurrence times were 36, 41, 51, and 96 months. Local recurrence and lung metastasis were detected in two patients, local recurrence in one, and lung nodules in the fourth patient. At the time of relapse, the chemotherapy protocols were as follows: vincristine, actinomycin-D, adriamycin, and cyclophosphamide in two patients; vincristine, actinomycin-D, and epirubicin in one patient; and vincristine, actinomycin-D, and adriamycin in the last patient. In the cases with late local recurrence, one patient had a local spillage and one patient had regional lymph node involvement. Although the other patient had local spillage, regional lymph node involvement, and renal artery invasion, isolated lung recurrence was observed. Only one patient had progressive disease and is still under treatment, whereas the other patients died with disease. Major recurrence sites were both local and the lungs. All of the patients had regional features including spillage, regional lymph node involvement, and vascular or capsular involvement. Late recurrence in patients with Wilms' tumor is a poor prognostic factor and should be treated with an intensified regimen.