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Objective: The aim of this study was to evaluate the outcome of germ cell tumors in patients admitted to our center during a ten year period. Methods: In a retrospective descriptive study, patients with the pathological diagnosis of germ cell tumor (GCT) were included. All records were evaluated and patients followed by personal visit in clinic or phone call. Data regarding age, sex, tumor site, bio-chemical assay, pathology, treatment and outcomes were gathered. For qualitative variables we computed frequency and percentage and for quantitative variables, mean and standard deviation. Survival analysis was performed using Kaplan-Meier. All statistical analyses were performed by SPSS version16.0. Findings: Forty four patients consisted of 32 girls (72.7%) and 12 boys (27.3%). Their median age was 23 months. The most common pathological tumor types were 18 (40.9%) mature teratomas and 14 (31.8%) yolk sac tumors. Extra gonadal tumors were more prevalent (32 cases) and consisted of 21 (47.7%) sacrcoccygeal, 7 (15.9%) retroperitoneal, 2 (4.4%) mediastinal and 2 (4.4%) cervical tumors. In gonadal tumors 9 patients had ovarian and 3 patients testicular involvement. Staging at the time of diagnosis revealed stage one in 23 (52.3%) cases. All patients were treated surgically and the most common procedure was total resection in 41 (93.2%) patients. Fifteen (34.1%) patients received chemotherapy. In follow-up 31 (77.5%) patients were in complete remission, 9 (22.5%) had died, and 4 cases did not appear to follow-up visits. The median survival was 16 months (IQR 4-49 months). The highest mortality rate was found in patients with yolk sac tumors (8 of 13 cases). Conclusion: The patients with extra-gonadal GCT and a high AFP level have the worst prognosis and lower survival rate. Combination of surgery and chemotherapy can lead to a better prognosis. [ABSTRACT FROM AUTHOR]

Objective: Temporary vesicostomy is a urinary diversion procedure for patients with upper urinary tract (UUT) dilatation, secondary to bladder outlet obstruction or dysfunction. The aim of this study was to evaluate our experience in children undergoing such diversion, analyzing its efficacy to prevent urinary tract infection (UTI), improve or resolve hydronephrosis, stabilize or improve kidney function and restore the health of UUT. Methods: In this retrospective study, patients who had vesicostomy by Blocksom technique due to bladder outlet obstruction or dysfunction were evaluated in Mofid Children's Hospital (in Tehran) from March 2007 to March 2012. The reason for applying this procedure was failure in clinical treatment. Data regarding gender, age, diagnosis, time of any surgical intervention, associated anomalies, primary/secondary complications and mortality were collected using a questionnaire, and evaluated by giving a grade that ranged from 0 (worst) to 10 (best) based on Lickert's scale. Findings: From a total number of 53 patients, (88.7% male and 11.3% female) with a mean age of 225 days, 66% had posterior urethral valve and 16 (30%) neurogenic bladder. UTI was present in all cases, hydronephrosis in 52 (98.1%), and vesico-ureteral reflux only in 45 (84.9%) patients. Valve ablation was performed in 17 cases, and clean intermittent catheterization in14 patients which were unsuccessful. We performed vesicostomy in all patients. Mortality rate was 7.5%. Vesicostomy was closed in 35 patients. Cure rate was 85% in UTI, 82.7% in hydronephrosis, 80% in VUR, and 86.5% in kidney function. Conclusion: Vesicostomy is a simple procedure that protects upper urinary tract, decreases hydronephrosis, and improves kidney function. The procedure is well tolerated and reversible, with less complication and should be considered in children in whom conservative and medical treatment has failed. [ABSTRACT FROM AUTHOR]

BACKGROUND The purpose of this study was to evaluate the characteristics, management, and outcomes of disc battery ingestion in children. METHODS We reviewed the medical records of children admitted to Mofid Children's Hospital due to disc battery ingestion from January 2006 to January 2010. Clear history, clinical symptoms and results of imaging studies revealed diagnosis of disc battery ingestion in suspected patients. The clinical data reviewed included age, gender, clinical manifestation, radiologic findings, location of disc battery, duration of ingestion, endoscopic results and surgical treatment. RESULTS We found 22 cases (11 males and 11 females) of disc battery ingestion with a mean age of 4.3 years (range: 9 months to 12 years). Common symptoms were vomiting, cough, dysphagia, and dyspnea. The mean duration of ingestion was 2.7 days (4 hours to 1.5 months). A total of 19 patients had histories of disc battery ingestion, but three cases referred with the above symptoms, and the batteries were accidentally found by x-ray. Only three cases had batteries impacted in the esophagus. Twelve batteries were removed endoscopically, 6 batteries spontaneously passed through the gastrointestinal (GI) tract within 5 to 7 days, and 4 patients underwent surgery due to complications: 3 due to tracheo-esophageal fistula (TEF) and 1 due to intestinal perforation. There was no mortality in our study. CONCLUSION Most cases of disc battery ingestion run uneventful courses, but some may be complicated. If the battery lodges in the esophagus, emergency endoscopic management is necessary. However, once in the stomach, it will usually pass through the GI tract. [ABSTRACT FROM AUTHOR]

Objective: Successful results after one-stage trans-anal pull-through (OSTAPT) operation for Hirschsprung's disease (HD) depend on accurate identification of the aganglionic segment in intraoperative frozen section (FS). Misinterpretation of the findings of the rectal biopsy is an anxiety- evoking pitfall for the surgeon. This study aims to describe our experiences in comparing results of FS and permanent Section (PS) rectal biopsies in children with HD who were candidates for OSTAPT in a single-step operation. Methods: Subjects under the age of 14 years, admitted from March 2000 to July 2008 in a university-affiliated children's hospital for open rectal biopsy to diagnose HD were included in the study. Ml biopsies were taken 2-3 cm above the dentate line. 210 specimens of full-thickness rectal biopsy were obtained for both frozen section and permanent biopsy from all patients^ examined by two well experienced pédiatrie pathologists for ascertaining the presence of ganglion cells, and the results were compared. Analysis was performed by SPSS Software version 11.5. Findings: Two-hundred one infants and children underwent FS rectal biopsy to exclude HD. Positive results were seen in 63.8% of the specimens examined as PS and in 58.3% of FS samples. 93.9% of positive results in FS studies were confirmed by PS studies. 6.1% of FS reports were false positive and 21,7% were false negative (P<0.001). The sensitivity of FS was 85.8% and specificity 90.2%. Positive predictive value (PPV) was 93.9% and negative predictive value (NPV) was 78.3% in FS studies (P<0.001). The accuracy of FS was 80.4%. Conclusion: Although FS of the rectal biopsy is useful in defining the aganglionic segment during operation, according to this study, it cannot be used as the sole base fer perfetntingprfftiary pull» through operation before the results ofthe permanent sectionare önfcand, [ABSTRACT FROM AUTHOR]

Objective: Acute scrotal conditions are a common clinical setting that present with pain and swelling of the hemiscrotum. The aim of our study has been to evaluate the findings in boys operated on acute scrotum. Methods: A descriptive study was conducted on 100 patients with acute scrotum admitted to Mofid Children's Hospital from March 1993 to March 2007. Data included history, age, primary symptoms, definite diagnosis, side involvement, paraclinical tests, imaging modalities, medical or surgical management and type of the surgery. Findings: Diagnosis was made mainly by clinical signs and symptoms and surgical exploration. Torsion of testis (n=31) was the most common cause of acute scrotum followed by incarcerated inguinal hernia (n=30), torsion of testicular appendage (n=27), epididymo-orchitis (n=7), idiopathic scrotal edema (n=4) and hematocele (n=1). Most (34%) of the patients were in the first year of life and the mean age was 5.4 years. The commonest signs were pain and swelling (62%) followed by pain, swelling and redness (21%) and pain alone (16%). 83 patients consisting of 31 with torsion of testis, 14 with torsion of testicular appendage, 30 with incarcerated hernia and 7 with epididymo-orchitis underwent surgical exploration after careful physical examination. 10 of 31 patients with torsion of testis had orchiectomy and orchiopexy of contra-lateral testis and the rest had detorsion and bilateral orchiopexy. 80% of patients were referred to the hospital after 12 hours of clinical onset of symptoms. Conclusion: Early exploration of scrotum based on careful physical examination excludes the risk of misdiagnosis by diagnostic procedures and unnecessary delay by diagnostic techniques. Exploration of scrotum is a relatively safe and simple procedure with good cosmetic results, it also allows an accurate diagnosis to be made. [ABSTRACT FROM AUTHOR]

Background: Macrostomia as a rare facial deformity is classified among facial clefts. It originates from failure in union of maxillary and mandibular prominences of first brachial arch during 7th embryonic week. Case Presentation: We report a case of bilateral macrostomia (bilateral lip cleft) in a female newborn as a sole entity without other skeletal and facial deformities. The cleft was repaired by a simple linear triangular flap using extra oral landmarks to locate lip commissures. Patient was followed through a six-month period. Acceptable results were gained in mouth appearance as well functional aspects. Conclusion: Commissural repair through a linear flap can result in minimal visible scar with satisfying results in both esthetics and functional aspects. [ABSTRACT FROM AUTHOR]

The study was a prospective analysis of collected data to compare the effect of the insertion angle (parallel or oblique relative to the upper-end plates) of thoracic pedicle screws on postoperative thoracic kyphosis in patients with idiopathic thoracic scoliosis. This study was conducted on idiopathic adolescent scoliosis (AIS) patients. Two groups of patients were defined according to the insertion angle of thoracic pedicle screws in relation to the upper-end plate of the corresponding vertebra, the insertion angles being either oblique or parallel. Seven consecutive AIS patients were compared in each group. Pre- and postoperative standing X-rays were obtained. Cobb's angles, sagittal parameters such as thoracic kyphosis (TK) (T1-T12) and lumbar lordosis (LL) (L1-S1) were all measured. The average of the main curve or compensatory curve corrections showed no significant differences between the two groups. From a sagittal profile perspective, the mean TK in the oblique screw group was 39.3 ± 11.5 preoperatively, which was reduced to 30.9 ± 6.41 after surgery. The mean TK in the parallel group was 36.4 ± 14 preoperatively and 42.1 ± 13.9 postoperatively. There was a significant reduction in TK in the oblique screws group compared with the second group ( P  = 0.038). The insertion angle of pedicle screws had no significant effect on Cobb's angle and lumbar lordosis changes. However, oblique insertion of thoracic screws decreased thoracic kyphosis postoperatively, which could be considered a negative point. As such, the insertion of thoracic screws in a caudal direction should not be routinely used because of its lordogenic effect., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Ovarian masses represent a range of pathology from benign cyst to highly aggressive malignant tumors. It has been estimated that gynecologic malignancy account for approximately 2% of all types of cancer in children, 60-70% of these lesions arise in the ovary., Methods: All ovarian masses which were resected or biopsied in Mofid Children's Hospital from 2002 to 2012 were reviewed retrospectively. Patient's age, presenting symptoms, surgical procedures, pathological diagnosis, postoperative treatment, and outcome were obtained from medical records., Results: Fifty-seven girls (aged 40.2±57months with the range of 1 day to 15 years) underwent different types of ovarian operations (24 salpingo-oophorectomies, 10 oophorectomies, 21 ovarian cystectomies, and 2 ovarian biopsies). 50 children had unilateral ovarian mass (49.1% right and 38.6 left, respectively). The most common presenting symptoms were acute abdominal pain in 46%.Twenty one (37%) of our patients had ovarian torsion. Four (7%) patients had benign tumors, and 8 (14%) had malignant tumors. There were no age differences between those with benign type (8.2±2.6years) and malignant tumors (6.1±5.3years) (P=0.683)., Conclusion: Ovarian tumors are rare in children. Most are benign, in children presenting with acute abdominal pain, ovarian mass particularly neoplastic tumors should be suspected. An important proportion of these patients may require postoperative chemotherapy.

Background: Total colectomy is used in children with total colonic aganglionosis, Ulcerative colitis (UC) and familial adenomatous polyposis (FAP). The purpose of this study was to maintain ileocecal valve and rectal-sparing surgery for the prevention of fecal incontinence in these children., Methods: From1990 to 2011, 14 children with diagnosis of UC, FAP and Hirschsprung's disease were operated. Total colectomy was done with the preservation of patch of cecum with ileocecal valve and half of the rectum with ileocecorectal anastomosis. Distal ileum designed as S shape pouch and ileocecal valve were preserved. In Hirschsprung's disease, posterior rectal myotomy was established. The data were collected and analyzed., Results: The mean age of the patients was 54 months (ranged from 2 months to 18 years). Ten patients were male. Among 14 patients, Hirschsprung's disease, ulcerative colitis and FAP were seen in 10, 3, and one case, respectively. They were followed up annually. Clinical and endoscopic examinations were performed to evaluate the function of ileocecorectal anastomosis. They followed from 2 to 24 years. At first year, the patients experienced four to six bowel movements during the day and one at night. This frequency decreased over time. The main postoperative complications included recurrent enterocolitis (n=2), perianal fistula (n=2). Only 2 patients were suffering from some degree of fecal soiling., Conclusion: The results show that the Ileocecal patch- low rectal anastomosis in total colectomy leads to low complications and prevent fecal frequency and incontinence. It also increases absorptive function of ileum in children.

Background: Total colonic aganglionosis (TCA) is present in 4% - 5% of the patients with Hirschsprung's disease and has a high surgical mortality rate between 13% - 23%. Diagnosis and treatment of TCA is still a major challenge for pediatric surgeons. Many techniques with several advantages and disadvantages were established for its treatment. We have performed State's pull-through as total colectomy and ileo-proctostomy with long posterior rectal myotomy in TCA and severe dysmotility disorders., Methods: In this retrospective study, 13 patients with TCA were evaluated from 1992 through 2012 in two pediatric surgery centers, which consisted of 10 TCA, one intestinal neuronal dysplasia (IND), and two chronic intestinal pseudo- obstruction syndrome (CIP). All patients underwent total colectomy, resection of part of the involved small intestine, and rectal anastomosis in one layer with 4/0 vicryl with long posterior rectal myotomy. All patients had preoperative barium enema and rectal biopsy for diagnosis. Leveling ileostomy was performed in 12 patients and mid-jejunostomy in one patient. In two of 13 children, proximal diverting loop ileostomy was established after definitive operation. In three patients, trans-rectal myotomy was needed two weeks after the initial operation., Results: All patients were females with the age ranging from six months to six years. State's pull-through was carried out for all (the mean age of definitive procedure was 5.1 months). Rectal biopsy of 10 patients reported no ganglion cells of which one had extended aganglionosis to mid- jejunum, one had IND, and two had ganglionicbowel bowel with clinical presentation of CIP. The follow- up period was from six months to 10 years (the mean follow- up period was 10 months). There were no significant complications in 12 patients except episodes of diarrhea and severe dehydration which needed hospitalization. One patient with mid- jejunum aganglionosis had severe failure to thrive and needed repeated hospitalization for parenteral nutrition. All patients had acceptable bowel function following operation (two to six times a day). Now, five of the 13 patients are above the age of toilet training, having voluntary bowel movement with little or no medication (Leopromid)., Conclusion: State's pull-through is recommended in all cases of TCA and severe dysmotility problems of the colon. This technique is less difficult to perform, and avoids the complications and disadvantages of removal of the rectum and has satisfactory results.

Insufficient control of post-thoracotomy pain can produce breathing dysfunction and long term staying in neonatal intensive care unit (NICU). It can increase the incidence of pulmonary complications such as atelectasis, pneumonia and respiratory failure. The aim of this study was to determine the analgesic effect of continuous extrapleural nerve block, using ropivacaine, in neonates younger than 7 days old with esophageal atersia (EA) and the incidence of atelectasis and duration of hospitalization in NICU. For this purpose, from February 2007 till January 2009 in Mofid children's hospital, 68 neonates under 7 days old whom were candidate for thoracotomy due to esophageal atresia were, randomly divided into two groups in a controlled clinical trial. The cases received extrapleural infusion of ropivacaine 0.5% (0.1 mL/kg/h for 48 h) and controls received acetaminophen 20 mg/kg three times a day via the rectal route. Hemodynamically unstable patients and those who suffered from hospital infections were excluded from the study. After the surgery, all patients had spontaneous breathing without endotracheal tube and stable hemodynamic in NICU. Pain level was determined for each neonate, based on the neonatal infant pain scale (NIPS) grading. The incidence of atelectasis in the first 48 h after operation and throughout the NICU staying were also determined. Results showed that there were no significant difference in the mean age, sex proportions and mean weight between the two groups. The mean pain score in the group received ropivacaine (1.9 ± 0.7) was significantly less than the control group (5.2 ± 0.6) (p < 0.001). Five percent of cases (n = 1) and 100% of the control group (n=20) had pain scores equal or greater than 3 (p < 0.001). The incidence of atelectasis among cases was less than the control group (35% vs. 65% respectively; p = 0.58). Duration of hospitalization in the case group (12 ± 5.6 days) had no significant difference from the control group (13.6 ± 4.8 days) (p = 0.3) In conclusion, the results showed that continuous extrapleural infusion of ropivacaine reduces the pain noticeably and atelectasis relatively, after thoracotomy in neonates younger than 7 days suffering from EA, compared to the acetaminophen group.

Background: For many years, subcutaneous therapeutic port system was known as a major route to access central veins. However, significant complications have been reported through recent years. One of the most important complications of subcutaneous port implantation is skin necrosis. In order to decrease this complication, we would like to introduce subpectoral fascia port implantation through this study., Methods: Five hundred and twenty four patients with a variety of neoplastic diseases underwent port implantation, from March 2003 to March 2008 (60 months). All suitable size catheters were put in the superior vena cava through the internal jugular vein under general anesthesia. The ports were placed in the subcutaneous pocket (SCP group) in 342 patients and in the subpectoral fascia pocket (SPFP group) in 182 patients. Data were analyzed using Chi-square test and survival analysis for time (Kaplan-Meier)., Results: A total of 538 devices were placed for 524 patients in two groups (14 patients received a second device after removal of the first one, due to failure of the first implantation). Mean follow-up period was 508 days (8 - 2025 days).Common complications observed in the SPFP group were as follows: wound infection (7 cases, 3.8%), catheter obstruction (7 cases, 3.8%), catheter displacement (6 cases, 3.2%), port related infection (5 cases, 2.7%), and pocket hematoma (2 cases, 1.1%).Common complications observed in the SCP group were as follows: catheter displacement (12 cases, 3.5%), skin necrosis (11 cases, 3.21%), port exposure (9 cases, 2.6%), port related infection (8 cases, 2.3%), catheter obstruction (8 cases, 2.3%), and port rotation (3 cases, 0.9%)., Conclusion: The results showed that port implantation in the subpectoral fascia pocket had a lower rate of skin complications than the subcutaneous pocket implantation. According to this study, this procedure was not complicated by skin necrosis over the port, port exposure or port rotation.

Background: Wilms' tumor (nephroblastoma) is the most common renal malignancy of childhood. The aim of the study was to evaluate the characteristics of Wilms' tumor and the results of combined modality treatment obtained in our center in Tehran., Methods: Fifty-five patients diagnosed as having Wilms' tumor were studied in the period between February 1992 and March 2002. Demographic features, mode of presentation, associated anomalies, the stage of tumor, histopathologic results, and the survival rates were evaluated., Results: Of these 55 patients, 31 were males and 24 were females (M/F = 1.2). The mean age at the time of diagnosis was 45.2 months. The distribution of 54 operated patients according to the surgical stage was: stage I 32.7%, stage II 16.36%, stage III 38.1%, stage IV 9%, and stage V 1.8% (one patient (1.8%) has not been operated). Favorable histology was diagnosed in 54.5% and unfavorable histology in 43.6% of the patients. The patients were treated according to National Wilms' Tumor Study protocols. The relapse-free and overall 4 years survival rates were 71% and 86%, respectively., Conclusion: As a developing country, with similar relapse free and overall survival rates to National Wilms' Tumor Study, our institution showed an improvement in the treatment of patients with Wilms' tumor in recent 10 years, but with more adaptation to the National Wilms' Tumor Study treatment protocols better optimum results seem to be achievable.

Myelolipoma is a benign tumor originating from hematopoietic and adipose tissue. This tumor usually develops in the adrenal gland, but rare presentations of extraadrenal myelolipoma are also reported, which usually arise from the presacral region. Herein, we present a 6-year-old girl with myelolipoma with generalized involvement of her abdomen, pelvis, chest, and retroorbital region.