Adrenocortical tumours in children: a review of surgical management at a tertiary care centre.

BACKGROUND: Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management. METHODS: Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively. RESULTS: The median age of 24 children was 78 months. Fourteen patients had adrenocortical carcinoma, nine had adrenocortical adenoma and one had neuroendocrine differentiation of ACT. Endocrine dysfunction was noted in 79% of the patients. Five patients had preoperative chemotherapy but none had a decrease in tumour size. Transabdominal approach was used in all but two patients who had thoracoabdominal incision for excision of giant tumours and ipsilateral lung metastases. Two patients had visceral excision to achieve R0 resection. Five patients, four of whom had spillage and one with partial resection died of widespread disease. Two patients with stage 4 adrenocortical carcinoma are still on chemotherapy. All patients with stage I-III disease who had total excision without spillage (n = 17) are disease-free for 2-170 months. CONCLUSIONS: Our results show the importance of excision in ACT without spillage for survival. However, multicentre prospective studies should enhance the knowledge of children about ACT and develop alternative therapies for stage III and IV cases.

Revisiting long-term prognostic factors of biliary atresia: A 20-year experience with 81 patients from a single center.

BACKGROUND/AIMS: The present study aimed at investigating the long-term outcomes and prognostic factors of patients with biliary atresia (BA) diagnosed and followed at a single center. MATERIALS AND METHODS: Patients with BA treated during 1994-2014 at a large-volume pediatric tertiary referral center were reviewed retrospectively with regard to demographic, clinical, laboratory, and diagnostic characteristics for identifying the prognostic factors and long-term clinical outcomes. RESULTS: Overall, 81 patients (49 males, 32 females) were included. Mean age at diagnosis was 73.1±4.7 (median: 64) days. Of the patients included, 78 patients (96%) underwent a portoenterostomy procedure. Mean age at operation was 76.8±4.7 (median: 72) days. The surgical success rate was 64.8%. A younger age (either at diagnosis or surgery) was the only determinant of surgical success. The 2-, 5-, and 10-year overall survival (OS) rates, including all patients with or without liver transplantation, were 75%, 73%, and 71% respectively, whereas the 2-, 5-, and 10-year survival rates with native liver (SNL) were 69%, 61%, and 57%, respectively. Mean follow-up duration was 9.4±7.5 years. Successful surgery, presence of fibrosis and/or cirrhosis on the liver pathology, and prothrombin time [international normalized ratio (INR)] at presentation were independent prognostic factors for both OS and SNL. CONCLUSION: A younger age at diagnosis is strongly associated with surgical success in BA. Surgical success, the prothrombin time (INR) at presentation, and liver pathology are independent prognostic factors affecting the long-term outcomes in patients with BA. Therefore, timely diagnosis and early referral to experienced surgical centers are crucial for optimal management and favorable long-term results in BA.

Evaluation of surgically treated breast masses in children.

Dogan G, Soyer T, Ekinci S, Karnak I, Çiftçi AÖ, Tanyel FC. Evaluation of surgically treated breast masses in children. Turk J Pediatr 2017; 59: 177-183. We aimed to define the diagnostic and treatment characteristics of breast masses among female children who had undergone surgical treatment in order to figure out which children should be followed conservatively or treated surgically. We reviewed retrospectively 64 female patients operated for breast masses under the age of 18 years between 1977 and 2013. Patient demographics, symptoms, physical examination findings, size of the mass and its relation with menstrual cycle, follow-up period before and after surgery, diagnostic characteristics, laboratory analysis, diagnosis before and after surgery, indications for surgery, histopathological characteristics of the mass, postoperative complications, and recurrence rate were recorded. Histopathologic investigations among 41 girls with complete hospital records showed that 26 had fibroadenoma (FA; 63.4%). Eleven girls among the other 23 cases with incomplete hospital records but with histopathological results have also had FA (47%). The majority of breast masses in childhood are FA, the frequency and duration of follow up is important. If there is a mass that will not regress, that continues to grow or does not reduce in size during follow up, and for those with family history of breast cancer to definitely exclude the possibility of malignancy, surgical treatment may be appropriate.

Sclerotherapy with bleomycin versus surgical excision for extracervical cystic lymphatic malformations in children.

PURPOSE: Sclerotherapy (ST) with bleomycin is an effective treatment for cervical cystic lymphatic malformations (LM) in children. However, its efficacy for treating extracervical cystic LM in children has not been investigated adequately. This retrospective study compares the efficacy of ST with surgery for treating extracervical cystic LM in children. METHODS: The subjects of this study were children treated for extracervical cystic LM at our hospital between 1970 and 2013. We evaluated retrospectively the hospital records of these children for age, gender, presenting symptoms, location of the lesion, radiological findings, treatments, complications, duration of hospitalization, and outcome. RESULTS: We analyzed the records of 70 children (M:F = 1:9) with a mean age of 52.57 ± 54.87 months (range 1-204 months). The number of children treated by surgery alone, ST alone, and surgery plus ST was 53 (77 %), 13 (18 %), and 4 (5 %), respectively. Surgery comprised total excision (n = 41), near-total excision (n = 9), partial excision (n = 6), and incisional biopsy (n = 1). The complication and recurrence rates were lower, the complete response rate was higher, and the length of hospitalization was shorter in the ST group than in the surgery group (5 vs. 15 % and 8 vs. 17 %, respectively, p < 0.05; 91 vs. 77 %, respectively, p = 0.05; and 2.42 ± 1.67 vs. 13.57 ± 16.24 days, respectively, p = 0.03). CONCLUSION: ST is as safe as surgery for extracervical macrocystic or mixed LMs in children, but is much more effective with higher success rates and lower recurrence rates. Thus, ST provides a cost-effective and appropriate mode of treatment for children with extracervical cystic LM.

Recurrent hemoptysis and a mass in the thorax in an infant: the split notochord syndrome.

Split notochord syndrome is a rare group of developmental abnormalities caused by abnormal splitting or deviation of the notochord clinically resulting in the duplicated bowel associated with vertebral anomalies. We report on a case of 11-month-old female infant with mediastinal hyperechogenic cyst and intestinal duplication cyst associated with T5-T6 hemivertebrae, scoliosis, and nonfusion of posterior part of T6 vertebrae, presenting with severe hemoptysis and hematemesis. The cysts were surgically removed, and histopathologic analysis revealed that the mediastinal cyst was lined by gastric mucosa and intestinal one was lined with gastric mucosa including ectopic pancreatic tissue. After removal of the lesion the patient made an uneventful recovery and shows no signs of long-term pulmonary sequelae.

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.

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.

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.

The management of primary nonparasitic splenic cysts.

Primary nonparasitic splenic cysts (PNSC) are rare and their management in children has been controversial. We conducted this study to discuss various treatment modalities. The medical records of patients with PNSC (1991-2008) were evaluated retrospectively, including age, sex, history of trauma, presenting symptoms, physical examination and radiological findings, therapeutic approaches, and outcomes. Six patients, between 3 to 12 years of age with a male/female ratio of 2, were included. The presenting symptom was abdominal pain in all but one asymptomatic patient. Physical examination findings were unremarkable in all except for palpable spleen in two patients. Cyst sizes ranged from 3 x 4 cm to 10 x 12 cm. The patients were treated with aspiration-sclerotherapy (n=2), total splenectomy (n=2), partial splenectomy (n=1), and cyst excision (n=1). The pathological diagnoses were epidermoid cyst (n=5) and lymphangioma (n=1). The postoperative course was uneventful except for postsplenectomy fever (n=1), recurrence (n=1) and residual cyst (n=1). PNSC larger than 5 cm in diameter or those that are symptomatic should be treated surgically. Total splenectomy should not be done in children to avoid infectious postsplenectomy problems unless there is a mandatory condition like intraoperative bleeding. Aspiration-sclerosis is not recommended because of recurrence.

Presenting clinical features and outcome in intussusception.

OBJECTIVE: To evaluate if a correlation exists between the clinical and radiologic characteristics at presentation; and the success of conservative management, morbidity and outcome of patients with intussusception. METHODS: All patients (total 179) treated for intussusception in our unit between 1993 and 2003, were retrospectively reviewed to find out the effects of physical examination (general appearance, consciousness, body temperature, abdominal distention and tenderness, blood on rectal examination), laborat (leukocyte count) and radiologic (air-fluid level on X-ray, free abdominal fluid on ultrasonography) findings on selection of first step therapeutic modality, conservative management (reduction with barium or air) success rate and surgical complication (serosal defect, intestinal perforation) rate. Additionally, the patients were subdivided into three subgroups as group A (patients for whom surgical management was performed primarily), group B (the ones who were operated after failure of reduction attempt with barium or air), group C (patients who had successful conservative reduction). These groups were compared within each other with regard to duration of nasogastric suction, antibiotic therapy; onset of oral feeding and duration of hospitalization. The chi-square, Kruskal-Wallis and ANOVA tests were used for the statistical analysis and p value less than 0.05 was considered to be significant. RESULTS: Incidence of selecting conservative treatment primarily was lower in patients with toxic appearance (p=0.02) and with free fluid on ultrasonography (p=0.007). Success rate of conservative treatment was lower in patients with moderate general appearance (p=0.000), lethargy (p=0.011), blood on rectal examination (p=0.004), air-fluid level on X-ray (p=0.039), free fluid on ultrasonography (p=0.001). Surgical complication rate was higher in patients with moderate general appearance (p=0.000), lethargy (p=0.007), air-fluid level on radiograph (p=0.009). In group A; the duration of N/G suction, antibiotic therapy and hospitalization was longer; the onset of oral feeding was later than the other two groups (p=0.000). In group B; the duration of N/G suction, antibiotic therapy and hospitalization was longer; the onset of oral feeding was later than group C (p=0.000). CONCLUSION: Toxic appearance, lethargy and air-fluid level on radiograph not only decrease the success rate of conservative management, but increase the surgical complication rate as well. Thus, these parameters deserve more importance in the management scheme. Reduction with conservative management is the most important factor which decreases the morbidity. Morbidity is lower in cases who are operated after failure of conservative management than the ones for whom surgery is performed primarily. Thus, conservative management should be tried for all patients unless there is a clear cut contraindication such as peritonitis and/or pneumoperitoneum.

Do radiologic studies correlate with each other and with surgical findings in intussusception?

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.

Endoscopic treatment of vesicoureteral reflux in children: our experience and analysis of factors affecting success rate.

AIM: To clarify the factors affecting the success rate of endoscopic subureteral injection (ESI) treatment of vesicoureteral reflux (VUR). METHOD: All patients who had ESI treatment for VUR at our unit between 1999 and 2002 were retrospectively reviewed with regard to age, gender, causes of VUR, reflux grade, type of injected materials, number of injections, results of cystourethrograms (VCUG), and outcome. The injected implants were gluteraldehyde cross-linked bovine collagen (GclBC), dextranomers in sodium hyaluronan (DiSH) and calcium hydroxylapatite (CH). A successful result was defined as absence of VUR confirmed by VCUG performed 3 months after the ESI procedure. Basic descriptive statistics were performed along with the chi(2) test (p < 0.05 was significant). RESULTS: 50 children (81 ureters) consisting of 27 girls (43 ureters) and 23 boys (38 ureters) with a mean age of 7.4 +/- 4.6 years constituted the study group. Grade III VUR was the most prominent grade (42%, 34 ureters) noted in all age groups. There was no significant difference between boys and girls with regard to the number of each grade of VUR. The success rate of first ESI procedure was 55.6% and was similar in each grade of VUR varying from 50 to 66%. Repeated injections have resulted in an overall success rate of 84%. No significant difference was noted between the age groups with regard to the success rate of ESI. Repeated ESI procedures were found to be unsuccessful in grade V VUR when compared to other grades (p < 0.05).There was a significant difference between primary reflux (76.5%, 62 ureters) and exstrophic (21%, 17 ureters) patients with regard to the grade of VUR and success rate of ESI. Exstrophic patients presented with a higher incidence of grade V VUR (41%) and with a lower success rate of ESI (64.7%). DiSH was the most commonly used agent (47 ureters) followed by CH (22 ureters) and GclBC (12 ureters). The success rates of the ESI procedure by each material were 91.5, 81.8, and 58.3%, respectively. No significant difference was noted between DiSH and CH. Low success rates by GclBCwere attributed to less usage of the material. 82% of the ESI procedures were done by surgeon A (49 ureters) and B (18 ureters) with a success rate of 96 and 72%, respectively. No significant difference was noted between A and B with regard to the success rate and type of injected material and the success rate in each grade of VUR (p > 0.05). No untoward effects were noted in short- and long-term follow-up (mean 4 +/- 1.2 years) of any patient. CONCLUSION: The ESI procedure provides a high success rate for the treatment of VUR which decreases in grade V VUR and presence of exstrophia vesica. Single injection of various materials has been found to be successful in most of the patients with grade II VUR, whereas grade IV and III patients have required repeated injections which have resulted in 100 and 94% success rates, respectively. Grade I VUR can be managed by close follow-up and appropriate antibiotic therapy without any surgical and/or endoscopic intervention. Patients presenting with grade V VUR should undergo open surgery if the first trial of ESI procedure results in failure as repeated injections have proved to be unsuccessful in this grade. The success rate of the ESI procedure does not seem to be affected by the type of injected material and different surgeons performing the procedure after achieving the learning curve.

Langerhans cell histiocytosis with involvement of nails and lungs in an adolescent.

Skin and/or pulmonary involvement occur frequently in Langerhans cell histiocytosis (LCH) whereas nail involvement is rare. Herein, we present a case of LCH with initial nail disease and subsequent lung involvement causing recurrent pneumothoraces. Systemic chemotherapy was applied and intrapleural bleomycine and thoracoscopic pleurodesis with talc were performed. Although prognosis is not satisfactory in LCH with recurrent pneumothorax and nail involvement, the patient is under follow-up with no evidence of skin lesions, nail involvement, and pneumothorax for the last 10 months.

Foreign body ingestion in children: an analysis of pediatric surgical practice.

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.

Histological response to injected dextranomer-based implant in a rat model.

To investigate the short and long term histopathological alterations caused by submucosal injection of dextranomers in sodium hyaluronan (DiHA) based on an experimental rat model. Sixty Sprague-Dawley rats were assigned into two groups as group I and II, each containing 30 rats. Totally 0.1 ml of saline solution and 0.1 ml of DiHA were injected into the submucosa of bladder of first (control) and second groups, respectively. Both group I and II were further subdivided into three other groups as Group IA, IB, IC and Group IIA, IIB, IIC according to the sacrificial period. Group IA and IIA, IB and IIB, IC and IIC rats (ten rats for each group) were sacrificed 3, 6, and 12 months after surgical procedure, respectively. Two slides prepared from injection site of the bladder were evaluated completely for each rat by being unaware of the groups and at random by two independent senior pathologists to determine the fibroblast invasion, collagen formation, capillary growth and inflammatory reaction. Additionally, randomized brain sections from each rat were also examined to detect migration of the injection material. The measurements were made using an ocular micrometer at 10x magnification. The results were assessed using t-tests for paired and independent samples, with P<0.05 considered to indicate significant differences; all values were presented as the mean (SD). Migration to the brain was not detected in any group. Significant histopathological changes in the DiHA injected groups were granuloma formation in 43.3%, foreign body type giant cells in 76.6%, inflammatory infiltration in 100%, and fibroblasts surrounding microspheres in 100% of the rat bladder. The interaction between granuloma formation and long-term tissue effects in children is still obscure. We emphasize that further prospective human (and/or animal) studies are required to clarify the long-term effects of granuloma formation with regard to clinical applications.

The association of pulmonary lymphangioleiomyomatosis with renal and hepatic angiomyolipomas in a prepubertal girl: a previously unreported entity.

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.

Portal vein thrombosis after splenectomy in pediatric hematologic disease: risk factors, clinical features, and outcome.

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 x 10(3)/mm(3) and 752.5 g, and 465.2 x 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.

An unusual penetrating injury in an infant: straight-pin migration from the back to the stomach.

Sharp foreign bodies reach into the abdomen frequently by ingestion and may lead to various complications including perforation. An 11-month-old infant is presented for straight-pin migration into the abdomen with penetration from the back and through the left diaphragm, spleen, and stomach. The infant was treated successfully with a well-organized diagnostic and therapeutic algorithm.

Paratesticular metastasis from Wilms tumor associated with a hydrocele.

Metastatic sites other than the lungs, lymph nodes, and liver are unusual for Wilms tumor (WT). Intra-scrotal metastasis is very rare. We report a 3-year-old boy with stage IIA WT, who experienced paratesticular metastasis 2 months after surgery for an abdominal recurrence. He had right scrotal hydrocele at initial diagnosis. The patient underwent right radical orchiectomy, and pathological examination revealed paratesticular WT metastasis. Intra-abdominal and peritoneal disseminated metastases followed. We considered that tumor cells spread through the patent processus vaginalis and grew at paratesticular space in hydrocele. One month after the end of 12 months of salvage chemotherapy and abdominal radiotherapy, the patient has no evidence of disease.