Evaluation of the removal reasons of totally implantable venous devices in children: a retrospective study.

Totally implantable venous access devices (TIVADs) increase the quality of life in children with hematologic and oncologic diseases or organ failures. The aim of this study is to determine the reasons for port removal. The port catheters, implanted and removed in patients between January 2000 and June 2013 were evaluated retrospectively. The patients were divided into two groups, whose port catheters were removed due to completed therapy (completed therapy group, CTG) and whose port catheters were removed because of a port catheter-related complications (complication group, CG). In the CG, the patients whose port catheters are removed for infectious reasons are investigated for whether there is a relationship with age, gender, body mass index (BMI), height and weight at the time of port implantation and removal. In total, 242 patients who underwent port implantation and removal were included in the study. The male to female ratio was 1.32/1 and the mean age of the patients was 9.4±4.9 years (0-24 year). Patients were enrolled in CTG (n=170, 70.2%), and CG (n=72, 29%). There is a positive correlation between BMI and infections (p < 0.05). In the CG, patients under steroid treatment had higher incidence of non-infectious causes than infectious causes (p < 0.05). Oppositely, non infectious complications were higher in steroid free patients (p < 0.05). There was no catheter related mortality in the entire study group. The hematological malignancies and solid tumors are the most common underlying primary disease in patients with port removal because of complications. Infectious complications are most common cause of port removal in children and despite other microorganism, fungi should be considered as a cause of catheter related infections.

Eccentric circummeatal based flap with limited urethral mobilization: An easy technique for distal hypospadias repair.

BACKGROUND: Hypospadias is a common congenital anomaly. Over 300 techniques have been described for repairing hypospadias. OBJECTIVE: Eccentric circummeatal based flap with combined limited urethral mobilization technique (ECMB-LUM) is a simple procedure to repair distal hypospadias with minimal complication rate. This study presents results of this technique, highlighting surgical pitfalls to achieve the best result. STUDY DESIGN: Medical records of patients with distal hypospadias operated on using the same technique between 1998 and 2011 were reviewed retrospectively. Age at surgery, position of meatus preoperatively and postoperatively, duration of urethral catheterization and hospitalization, early and late complications, previous hypospadias repairs, and secondary surgical interventions were evaluated. In the surgical technique an eccentric circummeatal based flap is outlined. The proximal part of the flap is dissected from the underlying urethra and Buck's fascia. If the flap is not long enough, the distal urethra is mobilized a few millimeters (Figure). The eccentric flap is sutured to the tip of the glans. The glans wings are approximated in the midline. A urethral catheter of 6 Fr or 8 Fr is passed and left in the bulbous urethra or the urinary bladder. Diverged limbs of corpus spongiosum are approximated on the urethra, then, the glans and skin of the penile shaft are sutured. RESULTS: Of the 171 consecutive patients operated on using the ECMB-LUM technique; 115 had coronal, 47 had subcoronal, and nine had glanular meatus. The mean age at surgery was 4.5 (1-17) years. Patients were hospitalized for 2.2 ± 0.7 days. Mean duration of urethral catheterization was 2.3 ± 0.5 days. All but eight patients had ECBF-LUM as primary repair. There were no early complications such as bleeding, hematoma, and wound infection. All patients voided spontaneously after catheter removal. Late complications were meatal stenosis, urethrocutaneous fistula, meatal regression, and glandular dehiscence (Table). These patients were treated using dilatation, fistula repair, meatoplasty, and secondary repair with the same technique, respectively. Eventually all patients had a vertical slit-like meatus on the tip of a natural looking glans. DISCUSSION: The most commonly used distal hypospadias repair techniques are glanular approximation, meatal advancement and glanuloplasty, Koff, Mathieu, Thiersch-Duplay procedure, tubularized incised plate repairs, and modifications of these techniques. Cosmetic and functional results and complication rates of ECMB-LUM technique are comparable with those of the commonly used techniques.

Factors affecting survival in neonatal surgery unit in a tertiary care university hospital during 26 years.

This clinical study was designed to evaluate mortality rate and the factors that may affect survival in neonatal surgery unit. Randomly chosen 300 (ß: 0.20) patients among 1,439 patients treated in neonatal surgery unit during years 1983 to 2009, were evaluated retrospectively. The patients were separated into three groups according to date of treatment; Group A: 1983 - 1995, Group B: 1996 - 2005 and Group C: 2005 - 2009. M/F ratios did not differ between non-survived and survived patient populations. Mortality rates were 37%, 22% and 13% in Group A, B, and C respectively (p < 0.001). Parenteral nutrition, maternal age, time until admission and gestational age did not affect mortality rate, however median age of newborn was lower in non-survived cases (1 day vs. 3 days, p < 0.001). Associating abnormality, low birth weight ( < 1,500 g), associating sepsis, need of globulin and requirement of respiratory support were determinants of lower survival (p < 0.001). The mortality rate for patients that underwent thoracotomy (42%) and laparotomy (41%) were higher than patients that underwent other operations (8%) and observation (10%) (p < 0.001). Diaphragmatic hernia had higher mortality rates than the other pathologies (p < 0.001). Survival rate is increasing to date in newborn pediatric surgery unit; it is independent from parenteral nutrition, maternal age, time to admission and gestational age however it is affected adversely by the age of patient, associating abnormality, low birth weight, presence of sepsis and requirement of respiratory support. Increase in survival could be related to various additional factors such as development of delicate respiratory support machines, broad spectrum antibiotics, hospital infection control teams, central venous catheters, use of TPN by central route, volume adjustable infusion pumps, monitoring devices, neonatal surgical techniques, prenatal diagnosis of pediatric surgical conditions and developments of environmental control methods in neonatal surgical units.

The efficacy of delayed surgery in children with high-risk neuroblastoma.

CONTEXT: Surgery is an important part of treatment in children with neuroblastoma; however, exact timing is unclear. Both initial and delayed surgery was suggested as the best by numerous studies. AIMS: Thus, we aimed to investigate the role of delayed surgery on 31 children with high-risk neuroblastoma. MATERIALS AND METHODS: Thirty-one children with high-risk neuroblastoma were enrolled into the study. STATISTICAL ANALYSIS USED: Statistical analysis was performed using Statistical Package for the Social Sciences (SPSS) for windows 10.0. RESULTS: There 'were 15 male and 16 female patients with a median age of 3.0 ± 3.2 years. Primary tumor site was adrenal in 27, non-adrenal in two, pelvic in one, and mediastinal in one patient. MYCN gene was amplified in four and non-amplified in 11 children on totally 15 children with available data. Lactate dehydrogenase was elevated in 30 children. The tumor volumes at diagnosis and before surgery in the whole group were 154.3 and 12.5 mL, respectively. The decline in tumor volume was statistically significant (P < 0.0001). Initial surgery was performed in three and delayed in 20 children, and eight children were inoperable. Surgical complication rate was 66.6% (two out of three patients) in initial surgery group; however, the rate was 15% (3 out of 20 patients) in delayed surgery group. The 5-year event-free survival and overall survival rates in the whole group were 44.8% and 50.8%, respectively. Primary tumor area control rate was 95% CONCLUSIONS: In conclusion, the delayed surgery with intensive chemotherapy and radiotherapy has been successful for primary control in high-risk neuroblastoma patients.

Enterobius granuloma: an unusual cause of omental mass in an 11-year-old girl.

Enterobius vermicularis (pinworm) is the only nematode that infects humans. It is one of the most common intestinal parasites. Pinworm commonly infests the terminal ileum and colon, and does not cause severe morbidity unless ectopic infection occurs. However, granulomatous lesions caused by ectopic Enterobius vermicularis infection may lead to unusual clinical symptoms and may be misinterpreted as malignant lesions. Herein, the authors present an 11-year-old girl with pinworm infection who presented with abdominal pain and an omental mass, with special emphasis on the diagnosis and treatment.

Composition of parenteral nutrition solution affects the time of occurrence but not the incidence of cholestasis in surgical infants.

Parenteral nutrition-associated cholestasis (PNAC) is one of the most important complications of parenteral nutrition (PN). This study was conducted to define the incidence, characteristics and precipitating factors of PNAC in infants treated with two different PN regimens in the pediatric surgery department. The records of infants who received two different PN regimens during January 2000-May 2008 (Group 1) and June 2008-May 2012 (Group 2) were reviewed retrospectively. Patients with and without PNAC comprised the study (Groups 1a-2a) and control groups (Groups 1b-2b), respectively. With Group 1 (n=109) and Group 2 (n=46) constituted as indicated, Group 1a (n=13), Group 1b (n=32), Group 2a (n=6) and Group 2b (n=8) were evaluated. The incidence of PNAC was 14.1% (n=22), and the type of PN regimen did not affect the incidence (14.6% vs. 13.0%, p=0.39). The duration of PN was longer in Group 1a than in Group 1b (34 ± 3 7 vs. 13 ± 10 days, p=0.01), and longer in Group 2a than in Group 1a or Group 2b (146 ± 148 vs. 34 ± 37 days, p=0.02, and 14.0 ± 5.9 days, p=0.002). PNAC developed later in Group 2a than in Group 1a (42.0 ± 24.6 vs. 9.0 ± 8.3 day, p=0.02). The lipid dosage was higher in Group 1a than in Group 1b (3.5 ± 0.7 vs. 3.45 ± 0.54 g/ kg/day, p=0.001) or Group 2a (2.2 ± 0.4 g/kg/day, p=0.01). The lipid dosage was also higher in Group 2a than in Group 2b (p=0.001). Two different regimens resulted in similar PNAC rates. The composition of nutrients in PN solution affects the time of occurrence but not the duration of PNAC. PNAC should be detected as early as possible and treated promptly.

An unexpected diagnosis in children with male phenotype and bilateral nonpalpable gonad: congenital adrenal hyperplasia with female genotype.

PURPOSE: Female pseudohermaphroditism is the most frequent form of ambiguous genitalia in children with congenital adrenal hyperplasia (CAH). However, a small group of children with complete urethral development in contrast to 46XX karyotype can be encountered. We aimed to define the characteristics of patients with 46XX CAH but having fully developed male external genitalia. METHODS: The records of 11 children with CAH and 46XX karyotype but having male phenotype, encountered from 1990 to 2012 were reviewed retrospectively. The age, presenting signs and symptoms, diagnostic studies, surgical procedures, early results and outcome were noted. All patients were evaluated by gender assignment team and the decision of the family was also taken into consideration during gender assignment. RESULTS: Eleven children (mean age 3.64 ± 3 years) (range 5 days-10 years) were enrolled. The main presenting signs were nonpalpable gonads (n = 7), hyperpigmentation (n = 2), jaundice (n = 1) and electrolyte imbalance (n = 1). All patients had bilateral nonpalpable gonads and male phenotype with mean phallus length of 4.5 ± 1.7 cm. Urethral meatus is located at normal position (n = 6) or hypospadiac (n = 5). Labioscrotal fusion was complete in all cases and they were classified as 4th (n = 3) or 5th (n = 8) degree of virilization according to Prader's classification. All children had CAH and 46XX genotype. Pelvic ultrasound (n = 8) and genitocystogram (n = 9) were used, and genitocystoscopy was performed (n = 6). Male gender was assigned in most (n = 10) and female gender in one. Corrective surgery could be performed in 10 patients. The surgical procedures were hysterectomy + bilateral salphingo-oophorectomy + vaginectomy (n = 9), chordee release (n = 3) and then second-stage (n = 2) or one-stage urethra repair (n = 1), and pure one-stage urethra repair (n = 1). One case underwent surrenalectomy before the diagnosis of CAH. Mastectomy (n = 1) and fistula repair (n = 3) were additional operations. Only one child could be undergone feminizing genitoplasty and another was lost to follow-up. CONCLUSION: Unfortunately, most of the children underwent surgery in adverse to genotype because of constituted sexual identity. Children with male phenotype and bilateral nonpalpable gonads should undergo promptly biochemical analyses for CAH and early chromosomal analysis.

A unique case of magnet ingestion with respect to presentation and management.

Magnet ingestion may lead to serious complications with delay in diagnosis and treatment. The forceful attraction between magnets, with gastric and/or intestinal wall entrapped between them, can cause injury through pressure necrosis. The radiological appearance of more than one magnet on X-ray can be easily misinterpreted as belonging to only one rod-like radiopaque foreign body, even if the magnets are located in different parts of the gastrointestinal tract, thus delaying the management up to the onset of emergent surgical complications. A 17-month-old female with ingestion of a pair of magnets is presented, together with introduction of the clinical picture and therapeutic approach, which differed from the other previously reported cases. The ovoid shape of the magnets, their localization in the gastrointestinal tract (leading to entrapped gastric and intestinal wall between them), absence of any complication, and the therapeutic approach of endoscopic retrieval are the main distinguishing features of this case from those previously reported.

Why gastric perforation occurs in patients with isolated esophageal atresia: more vulnerable stomach?

Gastric perforation (GP) in patients with isolated esophageal atresia (EA) is seen more than expected. The etiology of the perforation may be vulnerability of these immature stomachs. Gastrostomy operation should be performed by gentle handling. The volume of gastrostomy feedings should be increased incrementally. The early diagnosis of perforation is only possible with a high index of suspicion. The stomach should be visualized by gastrography with soluble contrast material as soon as the perforation is suspected.

The prognostic factors in children undergoing pulmonary metastatectomy.

A retrospective analysis was performed to determine the prognostic factors affecting survival in children who underwent pulmonary metastatectomy. Seventeen patients who underwent pulmonary metastatectomy between 2000-2006 were evaluated retrospectively by means of age, sex, primary diagnosis, time of metastasis appearance, number of nodules found on imaging examinations, type of management, surgical data, and outcome. Video-assisted thoracoscopic surgery (VATS) was used in 11 patients, and all patients underwent thoracotomy consequently. The nodule was composed of tumor cells in 13 (76%) patients and had positive surgical margins in 5 (38%). The time of metastasis appearance, number of metastases and completeness of the nodule excision did not affect survival (p=0.31, p=0.87 and p=0.56, respectively). Nodule size >1 cm was associated with dismal survival (p=0.008). Time elapsed until the diagnosis of pulmonary metastasis, number of metastases and the completeness of metastatectomy do not have an impact on survival. The only significant prognostic factor is the size of the largest metastatic nodule. The presence of a metastatic nodule >1 cm is associated with a worse outcome in pediatric patients. VATS is an adjunct method to thoracotomy in the surgical management of pulmonary metastasis in children.

Postoperative intussusception in children: a keen diagnosis in postoperative ileus.

We investigated the patients who developed postoperative intussusception after a variety of intraabdominal procedures in order to identify the differentiating features and facilitate the prompt recognition and management of this entity. Fourteen patients with postoperative intussusception following an abdominal surgery between 1993 and 2010 were analyzed retrospectively. The primarily applied surgeries were: repair of diaphragmatic hernia (n=3), choledochal cyst excision (n=2), extraction of surrenal neuroblastoma (n=2), Duhamel operation (n=1), colostomy closure (n=1), Nissen fundoplication with (n=1) and without (n=1) gastrostomy, gastropexy (n=1), gastrostomy and jejunostomy (n=1), and manual reduction of ileocolic intussusception (n=1), with a median duration of 135 minutes (120-240). Patients were reoperated on the 3rd day (2-16); intussusception was ileoileal in 11, and was manually reduced in 12 of all patients. Postoperative intussusception differs from other cases of invagination with respect to the pathogenesis, clinical presentation and therapeutic approach. The original operations are mostly the major and lengthy ones, with vicinity to the diaphragm. Awareness of this entity by surgeons and differentiation from other causes of postoperative ileus are obligatory for prompt recognition and management.

Management of acquired tracheoesophageal fistula with various clinical presentations.

PURPOSE: To determine the best therapeutic approach for acquired tracheoesophageal fistulae in childhood by evaluation of distinct clinical presentations requiring different surgical management based on our clinical experience. PATIENTS AND METHODS: Seven patients with acquired tracheoesophageal fistula seen between 1999 and 2010 were retrospectively studied with regard to the presenting findings, diagnostic evaluation, therapeutic approach, and outcomes. RESULTS: Five girls and two boys with a median age of 36 months (range, 2-156 months) were treated for acquired tracheoesophageal fistula. The presenting symptoms were respiratory difficulty (n = 3), coughing (n = 2), and dysphagia with coughing (n = 2), with a median duration of 30 days (range, 1-730 days). The etiologies were disc battery ingestion (n = 3), placement of endoesophageal prosthesis for caustic esophageal stricture (n = 2), corrosive ingestion with extensive burn (n = 1), and blunt chest trauma with subsequent emergency tracheotomy (n = 1). The site of the fistulae were proximal (n = 3) and middle (n = 1) trachea, left main bronchus (n = 1), and nearly the entire posterior wall of the trachea (n = 2). The patients were variously managed: conservatively with eventual spontaneous closure (n = 1), primary repair (n = 2), and colon interposition after cervical esophagostomy (n = 4) based on the clinical evaluation on admission and the follow-up status. Stenosis of the proximal esophagus (n = 2) and esophagocolonic anastomosis (n = 2) were the only complications encountered after treatment and were successfully managed with dilatation. CONCLUSIONS: The best therapeutic approach for acquired tracheoesophageal fistula can be determined with careful consideration of relevant parameters on admission, including medical history, presenting findings, etiology, and characteristics of the fistula, in addition to the clinical evaluation in the follow-up period. In general, conservative management should precede definitive surgical intervention both to allow for possible spontaneous closure and also to achieve optimal preoperative status. Primary repair or a staged surgical approach can be best selected by giving priority to the patient's airway security.

Papillary thyroid carcinoma does not have standard course in children.

BACKGROUND AND PURPOSE: Papillary thyroid carcinoma has a favorable outcome in children. Recent experience with young children cases with early recurrences, after the total thyroidectomy and excision of palpable lymph nodes, in contrast to usual course of disease in adolescent cases, prompted us to review our experience on papillary carcinoma surgery. MATERIALS AND METHODS: Sixteen children who underwent surgery for papillary carcinoma between 1997 and 2010 were included. The charts and surgery notes were evaluated retrospectively and age, sex, complaints and physical findings at presentation, past medical history, results of laboratory tests, imaging findings, aspiration biopsy, extent of disease, details of surgery, complications and postoperative course were noted. RESULTS: Male to female ratio was 3:1. The mean age was 10.9 years (range, 5-16). The presenting symptom was palpable swelling in the neck (n = 12) or asymptomatic nodule was detected incidentally (n = 4). Physical examination findings were palpable nodule (n = 7), lymphadenopathy (n = 6) or both (n = 4). Thyroid ultrasound (US) revealed nodule (n = 9), heterogenisity of the parenchyma and enlargement of thyroid (n = 9), and microcalcifications (n = 5). Fine-needle aspiration biopsy was performed in 12 patients and was suggestive for malignancy in most cases (91%). Pulmonary metastasis was detected at presentation in two patients and appeared after thyroidectomy in one patient. Complementary thyroidectomy was performed in nine patients because the total thyroidectomy with or without cervical lymph node dissection was the treatment of choice (n = 15). Iodine scan was performed 4 weeks later following thyroidectomy. Radioactive iodine ablation (RIA) therapy was given to 11 patients who had residual or recurrent disease. Postoperative complications were permanent hypoparathyroidism (n = 4), recurrent laryngeal nerve injury (n = 3), lymphorea (n = 1) and Horner's syndrome (n = 1). CONCLUSION: Total thyroidectomy and excision of affected lymph nodes is the current mode of surgical management for thyroid papillary carcinoma in children. However, especially in young children (<10 years), modified lymph node dissection should be added to total thyroidectomy in order to avoid leaving the residual tumor foci which can impair the efficacy of the ablation therapy.

The association of adrenocortical carcinoma and thyroid cancer in a child with Peutz-Jeghers syndrome.

Peutz-Jeghers syndrome (PJS) is a rare, dominantly inherited disorder characterized by gastrointestinal hamartomatous polyps, mucocutaneous hyperpigmentation, and an increased risk of cancer. We present a 16-month-old child diagnosed with PJS, who had distinguishing features compared with the previously reported cases with respect to her clinical presentation, associated malignancies, and genetic analysis. To our knowledge, this is the first report of adrenocortical carcinoma in association with PJS, as well as the first instance of associated thyroid cancer in a child with PJS. We briefly review the relevant literature and highlight the recent progress achieved in the investigation of the syndrome.

Late-onset hypertrophic pyloric stenosis: definition of diagnostic criteria and algorithm for the management.

BACKGROUND AND PURPOSE: Gastric outlet obstruction (GOO) may be caused by various congenital and acquired conditions in children. The authors report 11 cases of GOO caused by muscular hypertrophy of the pylorus, which was proven histologically in 7. They describe this entity as "late-onset hypertrophic pyloric stenosis (HPS)," define the diagnostic criteria of the disease, and produce an algorithm for its management. MATERIALS AND METHODS: The medical records of patients with GOO treated from 1999 and 2009 were retrospectively reviewed. Patients with infantile HPS and GOO secondary to corrosive ingestion or neoplasm were not included. Age, sex, family history, presenting symptoms and signs, radiologic methods and findings, type of management, histopathologic features, and outcome were noted for each patient. RESULTS: Eleven patients (4 male, 7 female) ranging in age from 2 to 8 years (mean, 3.6 years) were included in the study. The symptoms were nonbilious vomiting (n = 11), abdominal pain (n = 4), and weight loss (n = 2). Abdominal ultrasound (n = 6) and upper gastrointestinal contrast studies were obtained (n = 11). Gastroscopy revealed complete (n = 6) or partial (n = 5) obstruction of the pylorus. Balloon dilatation of the pylorus was performed in 5 cases and repeated in 3 cases. Conservative treatment was initially attempted in 11 patients. Two patients with chronic gastritis and Helicobacter pylori (n = 2) were treated with amoxicillin-clavulanic acid, clarithromycin, and lansoprazole. A Billroth I procedure was performed in 7 cases. Hypertrophied pyloric muscle was noted in 7 patients and chronic gastritis in 2. The postoperative course was uneventful. CONCLUSION: Hypertrophic pyloric stenosis is rarely seen after infancy. Analysis of our results and review of the literature prompted us to redescribe this entity as "late-onset HPS" and define the diagnostic criteria. Late-onset HPS is probably an acquired disease of unknown etiology. The management of late-onset HPS has been summarized in an algorithm, which will also be useful in the treatment of GOO in children caused by etiologies other than classical infantile HPS.

Sclerotherapy with bleomycin does not adversely affect facial nerve function in children with cervicofacial cystic lymphatic malformation.

BACKGROUND AND PURPOSE: Sclerotherapy with bleomycin sulfate (BS) is currently used in the management of cervicofacial cystic lymphatic malformations in children. Neurotoxic adverse effects of BS after intraventricular or intracavitary administration have been reported; however, the effects of intralesionally administered BS on the adjacent peripheral neural structures have not been previously investigated. The authors conducted a clinical experimental study to evaluate facial nerve function in children who have undergone BS sclerotherapy for cervicofacial cystic lymphatic malformation. MATERIALS AND METHODS: Twelve patients who underwent BS sclerotherapy for cervicofacial lymphatic malformation were included in the study. The hospital records were reviewed, and the following data were recorded: age at admission and at the time of motor nerve conduction study (MNCS) and electromyography (EMG) study, sex, time elapsed between sclerotherapy and the EMG study, and the outcome. The MNCS/EMG study was performed by neurologists blinded to the side of sclerotherapy. Bilateral facial MNCS and needle-EMG study of the orbicularis oris muscle on the treated side were performed. The previously treated and untreated sides constituted the study and control groups, respectively. In the MNCS, compound muscle action potential (CMAP) amplitude and distal latencies were recorded from the orbicularis oculi and orbicularis oris muscles on both sides, and needle-EMG of the orbicularis oris muscle was performed on the treated side. RESULTS: The male-to-female ratio was 2, and age at the time of sclerotherapy ranged from 1 month to 16 years (median, 19.5 months). The lymphatic malformations were located in the right submandibular (n = 5), left submandibular (n = 6), and in the right buccal (n = 1) areas. Bleomycin sulfate was injected 1 to 4 times, and the time elapsed between injections varied from 1 to 6 months. The results of sclerotherapy were excellent, with residual disease observed in only 1 patient. The MNCS/EMG study was performed 6 months to 10 years after completion of sclerotherapy, and ages of the patients at the time of the study ranged from 4 to 17 years. Side-to-side CMAP amplitude difference did not exceed 50% for orbicularis oculi and orbicularis oris muscles. The mean CMAP amplitude of orbicularis oculi and orbicularis oris muscles on the treated and untreated sides (1219.0 +/- 842.0 vs 1202.4 +/- 923.8 microV and 1866.3 +/- 911.5 vs 1921.0 +/- 910.0 microV, respectively) did not differ between groups (P = .76 and P = .80). Distal latencies recorded from orbicularis oculi and orbicularis oris muscles on treated and untreated sides (2.64 +/- 0.46 vs 2.68 +/- 0.47 milliseconds and 3.10 +/- 0.35 vs 3.10 +/- 0.25 milliseconds, respectively) also did not differ between groups (P = .71 and P = .80). Needle-EMG orbicularis oris muscle (n = 11) on the treated side showed normal findings at rest, and there was no spontaneous activity. During mild voluntary contraction, the amplitude and duration of motor unit action potentials were within normal limits except in one case. Interference patterns were also normal in all cases. CONCLUSION: Bleomycin sulfate did not adversely affect facial nerve function in children who underwent sclerotherapy for cervicofacial cystic lymphatic malformation when it was applied according to our protocol.

A rare type of renal cell carcinoma in a girl: hybrid renal cell carcinoma.

Hybrid renal cell carcinoma (HRCN) is a rare type of renal tumor with characteristic pathologic features, including oncocytic and chromophobe cellular content, and shows more favorable prognosis than renal cell carcinoma. The early-stage tumors show favorable outcome, and postoperative regular clinical and radiological follow-up is adequate in most cases. However, close follow-up is mandatory for tumors with histopathologically coexistence of squamous, papillary, and/or eosinophilic RCC component. This report describes a 12-year-old girl with a stage I HRCN who was closely followed after nephrectomy with no further treatment.

Neuroblastoma in Turkish children: experience of a single center.

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<0.001), abdominal primary tumor site (P<0.001), NBL subtype in histopathology (P=0.001), responsiveness to chemotherapy (P<0.001) positive or high Vanillyl mandelic acid levels (P=0.02) and male sex (P=0.008) were the determinants of poor prognosis. CONCLUSIONS: The survival rates in children with local disease are comparable with the results of developed countries; however, the results in children with advanced disease are still not satisfactory. To improve the outcome, especially in children with advanced disease, more effective chemotherapy regimens and molecular therapies should be investigated. Sharing the knowledge and capacity building to improve the treatment results in NBL are also critical for developing countries.

Management of unusually extensive esophagogastric corrosive injuries: emergency measures and gastric reconstruction.

Ingestion of a corrosive substance only rarely presents with life-threatening symptoms because of acute necrosis of the esophagus and/or stomach and necessitates emergency surgery. Once the patient is stabilized, a staged reconstruction of the alimentary tract is planned. The surgeon should be familiar with the various types of gastric reconstruction in conjunction with or without esophageal replacement. The authors report 2 illustrative cases, which presented severe symptoms after corrosive substance ingestion, to emphasize the important aspects of management of this condition. The reconstruction of the gastrointestinal tract in children is managed with a staged approach using various methods, including Hunt-Lawrence J pouch gastric substitution.

Gastric organo-axial malrotation coexisting respiratory symptoms.

Gastric malrotation is defined as a torsion of stomach around its short or long axis. It is a rare disease in childhood. Gastric malrotation may present either as a surgical emergency or as chronic abdominal symptoms. There is limited data about the respiratory symptoms associated with gastric malrotation. The aim of this study was to review the records of 14 children who presented with respiratory symptoms and diagnosed as gastric organo-axial malrotation. Between August 2005 and August 2007, 14 children diagnosed as having gastric organo-axial malrotation participated in this study. There were 11 boys and three girls with a mean age of 7.1 months. All patients were symptomatic. Presenting symptoms included wheezing in four patients, recurrent pneumonia in four, chronic cough in two, chronic cough and apnea in two, recurrent pneumonia and chronic cough in one, and chronic cough and failure to thrive in one. All of our patients had organo-axial gastric malrotation. Gastroesophageal reflux was found in nine patients (64.2%). Six patients were treated with surgery and antireflux medication and the remaining eight patients with antireflux medication. In conclusion, it is probable that gastric malrotation associated with massive or occult gastroesophageal reflux could be responsible for the respiratory symptoms such as chronic cough, recurrent pneumonia, wheezing, and apnea.