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Background: Esophageal atresia and tracheoesophageal fistula (EA/TEF) is a known correctable anomaly of the esophagus, and its prognosis depends on multiple factors. Studies investigating the EA/TEF post-operative outcomes among the Iranian population are limited to small sample sizes, and the current prevalence of coexisting anomalies and predictors of poor prognosis in the Iranian population is still unclear. Objectives: This study aimed to investigate the predictors of in-hospital mortality in neonates with EA/TEF at our center within a 12-year period. Methods: In this retrospective cohort study, we investigated neonates with EA/TEF admitted/referred to a tertiary referral center in Tehran, Iran, from March 2008 to April 2020. Neonates with chromosomal anomalies or age > 10 days at operation date were excluded. Baseline characteristics, associated anomalies, type of EA, and transmission distance were compared in the study population. We followed the neonates for incurring in-hospital mortality. Results: We included 233 neonates in the final analysis. The mean age at operation was 3.1 ± 1.7 days, and 111 (47.6%) cases were female. The most common EA type was type C (proximal esophageal atresia with distal fistula), with a prevalence of 94.4%. In this cohort, 23 (9.9%) cases had vertebral anomalies, anal atresia, cardiovascular malformations, trachea-esophageal fistula, renal and limb anomalies (VACTERL) association, and 29 (12.4%) cases died during the in-hospital course. Moreover, neonates with lower birth weight, gestational birth weight < 37 weeks, other coexisting anomalies, cardiovascular defects, and non-VACTERL anomalies were at higher risk of in-hospital mortality. In contrast, EA types and transmission distance did not increase the mortality risk. Furthermore, we measured a cut-off value of < 2575.0 g for birth weight to predict in-hospital mortality with 65.5% sensitivity and 61.3% specificity. Conclusions: Lower birth weight, prematurity (< 37 weeks), and coexisting anomalies, especially cardiovascular defects, were associated with an increased risk of in-hospital mortality in neonates after EA/TEF repair surgery.

Background: Covid-19 virus infection is a pandemic disease. Incidence of COVID-19 infection in children and neonates is less than adults. Neonates may be contaminated by close contact with the infected family members or environment. However, infection via transplacental transmission and breast feeding has not been proved yet. Hospitals face shortages of resources in the outbreaks. This becomes more important in underdeveloped countries. Methods: We have studied the neonates who underwent surgical interventions in emergent or urgent conditions. No specific protection measures were applied in the operating room and neonatal intensive care unit for patients without symptoms or positive history of contact, in an attempt to save the resources of the hospital. All the neonates and their families were followed up for two weeks. Results: Forty patients and their families were studied. At admission, our patients neither demonstrated symptoms of the disease nor gave positive history of contact, hence received surgical intervention in the ordinary protection. One patient suffered from respiratory problems in the post-operative period and tested positive for the Covid-19 infection. Six patients and Five family members became symptomatic during the follow-up. All of them tested negative for the diseases and recovered. Conclusion: Based on the results of our study, surgical interventions for the critical neonates without positive symptoms or history neither should be postponed nor be necessarily dependent on the application of the corona specific personal protection. This gains most significance in the peak of outbreak, especially in underdeveloped countries.

Background: Childhood cancer is the second leading cause of death in children. Approximately, 40% of children with cancer need to be admitted to the intensive care unit (ICU), which plays a key role in their treatment procedure and overall survival. Objectives: In this study, we aimed at evaluating the impact of ICU admission on pediatric patients’ survival and prognosis at Mahak Pediatric Cancer Treatment and Research Center (MPCTRC), Tehran, Iran. Methods: Amongst a total number of 2693 hospitalized patients who were referred to our center from March 2014 to September 2019, 674 patients younger than 14 years of age who were at least admitted once to the ICU were included in this study. All the collected data were analyzed using IBM-SPSS software version 22. Results: Totally, 48.96% of patients were female and 51.03% of them were male. The most frequent types of cancer were central nervous system tumors (33.23%) and Leukemia (18.99%). Additionally, 43.62% of admitted patients were in the 1 - 4 age group, with the mean age of 1.5 ± 0.30. The mortality rate in ICU was 40.20% and the overall survival (OS) rate was 29.5% ± 2.6. Moreover, the OS in children with solid tumors and non-solid tumors were 27.1% ± 5.5 and 32.6% ± 3.1, respectively. Conclusions: The results of this conducted study provide a complete report on the status of children admitted to the ICU ward in Mahak Hospital. Because of this fact that the most frequent type of cancer was CNS tumors and children with CNS tumors are usually admitted to the ICU in the late stages of their disease, the overall survival rate in our study was 29.80%, which is lower than other studies.

Renal ectopy is a rare phenomenon, which its diagnosis may be delayed due to an asymptomatic condition. The ectopic kidney may even have a normal functional state; however, because of occurring kidney blockade, the risk for kidney stones and even failure may be raised. In many cases, the diagnosis is completely incidental and is based on the request of radiography and sometimes, CT scan even for other reasons. In the therapeutic approach, in cases of kidney blockage, or urine backing up to the kidney, the surgical intervention is highly recommended by excisional removing the mass. Herein, we described a case of ectopic intrathoracic kidney initially presented with mild respiratory distress that was ultimately diagnosed by CT scanning and removed surgically.

Background: Hypospadias is a congenital anomaly on the penis, in which the meatal orifice opens ventrally and proximal to the tip of the penis. In this regard, two common treatment methods are tubularized incised-plate urethroplasty (TIP) and the Mathieu incised-plate (MIP) technique. The present study aimed to compare the early and long-term outcomes of TIP and MIP among patients with distal penile hypospadias. Objectives: The study was also to evaluate the postoperative functional outcome of hypospadias over a long-term follow-up. Methods: Fifty-nine patients were randomly selected and assigned to two groups (TIP (n = 31) and MIP (n = 28)). Demographic information, preoperative findings, and postoperative complications were collected from the two groups. The Hypospadias Objective Scoring evaluation (HOSE) questionnaire and uroflowmetry were obtained to evaluate the long-term outcome of hypospadias repair. Results: The success rates of the surgical TIP and MIP techniques were 71.0% and 82.1%, respectively. Postoperative complications in the TIP group were three (9.7%) distal UCF and four (12.9%) meatal stenosis. In the MIP group, two (7.1%) and three (10.7%) patients suffered from distal UCF and meatal stenosis, respectively. Moreover, 89.3% of the patients in the MIP group and 80% of the patients in the TIP group had acceptable HOSE. Regarding the uroflow rates in the MIP group, 12% and 58% of the participants were below the 5th percentile and above the 25th percentile, respectively. Concerning the uroflow rates of TIP, 32% of the patients were below the 5th percentile, and 18% of the participants were above 25th percentile. Conclusions: Although there have been some reports on the superiority of TIP, we found these two techniques at approximately equal levels with a slightly higher success rate for the MIP regarding the early outcomes. With the exception of the long-term outcomes in cosmetic and functional evaluation, MIP is superior to TIP.

Introduction: Chronic abdominal pain in children is mostly functional, but in association with alarm symptoms such as recurrent vomiting, it is necessary to determine proper tests for the diagnosis of probable underlying organic problems. Case Presentation: Four patients with chronic refractory abdominal pain and nonspecific gastrointestinal symptoms presented to our tertiary pediatric center. After thorough medical and psychological investigations and hepatobiliary scintigraphy, and calculating gallbladder ejection fraction, laparoscopic cholecystectomy was performed. One year after the surgery, they were relatively symptom-free and returned to their routine life. Conclusions: Biliary tract abnormalities should be considered as a probable cause of chronic abdominal pain in children. Hepatobiliary scintigraphy can provide promising results to help to identify the underlying causes of chronic abdominal pain in association with nonspecific gastrointestinal manifestations.

Introduction: The congenital intestinal web is a rare cause of small bowel obstruction classified as type I intestinal atresia. The second portion of the duodenum is the most prevalent site for these webs, whereas the jejunal web is rare. Case presentation: In this study, we present a very uncommon late presentation of the jejunal web in an 18-month-old girl complaining of chronic recurrent vomiting, constipation, and failure to thrive (FTT). After thorough investigations, the diagnosis was confirmed by the small bowel follow-through. The surgical procedure resulted in a significant improvement in patient’s quality of life, given the subsequent increase in her weight and height. Conclusions: Vomiting that occurs at any age is pathologic and requires careful history taking thorough clinical examination and evaluation, especially in patients treated with proton pump inhibitors such as omeprazole for a long time with a diagnosis of gastroesophageal reflux disease (GERD) without any response. In these patients, gastrointestinal abnormalities, including complete or incomplete obstructions such as malrotation and web, should be made in the differential diagnosis.

Background: Wilms’ tumor (nephroblastoma) is the major renal cancer in children. Objectives: The aim of this study was to assess the individuality of Wilms’ tumor and the consequences of management attained in our referral subspecialty center. Methods: In this study, we composed the data of children with Wilms’ tumor in 2 decades; 55 cases between 1992 and 2002 and 49 patients between 2006 and 2016 were diagnosed with Wilms’ tumor. Demographic characters, a form of presentation, tumor stage, related underlying disease, histopathology consequences, type of management, and the survival rates were assessed. Results: In the first decade, 24 patients were females and 31 were males (M/F = 1.2); in the other groups, 30 were females and 19 were males (M/F = 0.61). The mean age was 45.2 months at the time of diagnosis for the first group and the mean age was 36 months for the other group. In the first decade, the surgical stage after the operation was as follows: stage I (32.7%), stage II (16.36%), stage III (38.1%), stage IV (9%), and stage V (1.8%) who did not operate. In second decade, 49 patients were as follows: stage I (14.3%), stage II (40.8%), stage III (24.5%), stage IV (10.2%), and stage V (10.2%). In 54.5% of the first group, histology was favorable, and in 43.6% of the first group, histology was unfavorable; in the second group, 95.4% were the favorable type. The patients were managed based on protocols of the National Wilms’ Tumor Study. In the first decade, relapse-free was 71% and 4-year survival rates were estimated at 86%, and in the second decade, pulmonary metastasis was observed at 28.6%, liver metastasis in 2.3%, recurrence in 5%, and 4-year survival rates were estimated at 90%. Conclusions: This study demonstrated development in the management of children with Wilms’ tumor in recent 20 years, with comparable relapse-free and survival rates to the National Wilms’ Tumor study. But with more adjustment in treatment protocols, the superior outcome will be attainable.

Background Ureteric-pelvic junction obstruction (UPJO) is the most common cause of antenatal and neonatal hydronephrosis and its management remains controversial. While conservative management is advocated for all, this strategy puts a quarter of these patients at risk for possibly irreversible renal damage. Aim In this study, we compare functional and anatomic outcomes in newborns and infants less than 1 year of age with high-grade unilateral UPJO, following early surgical pyeloplasty (ESP) versus conservative management (CM). Materials and Methods This was a single center prospective interventional study. Infants referred to our tertiary care pediatric surgery clinic between September 2016 and September 2018 with UPJO were considered. To be included patients must have been less than 1 year old, lack of clinical symptoms, suffer from severe hydronephrosis as defined by Society for Fetal Urology (SFU) grades 3 or 4, and have affected kidney Split Renal Function (SRF) above 40%. Patients with bilateral disease, structural anomalies, or an abnormal voiding cystourethrogram (VCUG) were excluded. Anatomical and functional outcomes were measured and compared at 6 and 12 months. Results Fifty-six patients were assigned to receive either ESP (n = 28) or CM (n = 28). At 6 months Cortical thickness, polar length, and SFU indices were significantly lower in the ESP group, while none of the outcomes were significantly different between the two groups at 12 months. Despite the two groups not being different at 12 months regarding differential renal function (DRF), there was a significant decrease of function in the CM group compared to baseline. Conclusion When considering treatment options for infants with high-grade UPJO, it appears that ESP hastens improvement of anatomic and functional indices, while CM may lead to a significant deterioration in renal function.

The primary intrarenal neuroblastoma (IRNB) is a rare condition. Intrarenal neuroblastoma typically results from direct renal invasion from an adrenal neuroblastoma, but true intrarenal neuroblastoma originates either sequestered adrenal rests during the fetal life or intrarenal sympathetic ganglia. Clinical, radiological, and pathological correlation is very essential for diagnosis and appropriate management of this type of unusual cases. The distinction of this rare tumor from Wilms’ tumor is an important challenge since both tumors have major differences in prognostic and therapeutic response. We present a 3-year-old boy of primary intrarenal neuroblastoma with extensive abdominal and mediastinal mass, persistent hypertension, and disseminated intravascular coagulation (DIC).

Background: Hypospadias is one of the most common congenital genital anomalies in males that necessitates to be operated early in infancy (when 6 to 9 months old). On the other hand, hypospadias is a challenging field of pediatric urology with multiple reconstruction techniques. A perfect hypospadias repair is supposed to return urethral continuity with sufficient caliber, eradicate phallus curvature, and supply an acceptable appearance with low complications. Objectives: This study aimed to evaluate the outcomes of using onlay island flap technique in the repair of hypospadias with shallow urethral plate. Patients and Methods: In this prospective study within June 2012 to December 2013, we performed onlay island flap procedure to repair hypospadias with shallow urethral plate measuring less than 6 millimeter. This technique was selected for all types of hypospadiasis except subcoronal type. Nesbit’s dorsal plication procedure was established for chordee. In cases with very small glans, urethroplasty was performed without glansplasty. Results: Twenty three patients with mean age of 30 (range 10 - 60) months underwent onlay island flap repair; all had a shallow urethral plate < 6 mm, 3 had a very small glans, and 18 had chordee. Meatus was located in distal shaft in 5 cases, mid shaft in 8, proximal in 6 and penoscrotal type in 4 patients. Chordee was corrected with Nesbit’s dorsal plication in 16 cases. Complications were: meatal stenosis in 2 cases and urethrocutaneous fistula in 2 patients, all of which were repaired surgically. Mean follow up time was 13 (3 - 20) months. All cases that had glansplasty have excellent esthetic appearance. Conclusions: This technique offers acceptable results regarding meatal stenosis, urethrocutaneous fistula and esthetic outcome.

Background As children comprise a considerable proportion of our population, the importance of local epidemiologic research and geographic and racial differences can’t be disputed on childhood malignancies. Methods In this descriptive retrospective study, we extensively reviewed the medical records of patients younger than 15 years of age, diagnosed with solid malignant tumors, from 1996 to 2010, using the last version of International Classification of Childhood Cancers. Results In our study the order of incidence of solid malignancies was relatively similar to the other national studies, with lymphomas and Central Nervous System (CNS) tumors as the most common, followed by Sympathetic Nervous System (SNS) tumors, soft tissue sarcomas and renal tumors. The peak age of diagnosis was between 1 and 4 years old. In our study, the overall male to female ratio was 1.38, with a trend towards male dominance in the older age groups. We also observed a disturbing trend of childhood solid malignancies. The total number of cases almost doubled from 2009(54(6.9%)) to 2010(96(12.2%)) .This trend was particularly detected in CNS and SNS tumors. Further analysis showed that malignant CNS tumors had played a more pronounced role in this change. Conclusion Changes in trends of some tumor categories have illustrated a desperate need to further research in regional and national levels. Also the gathered data can be used to make more accurate programs for a better control of cancer and to help policymakers to allocate more evidence-based resource for hospitals.

Background: Laparoscopy is not an accepted procedure for complicated appendicitis in children for most pediatric surgeons. This procedure is associated with a higher incidence of postoperative abdominal abscess reported in some studies. Objectives: In this study, we investigated the security, efficacy and complications of laparoscopy in children with complicated appendicitis in Mofid Children's Hospital. Patients and Methods: From April 2010 to January 2013, we performed laparoscopic appendectomy (LA) in all cases of non-complicated and complicated appendicitis (including perforated appendicitis and localized or generalized peritonitis based on the operation findings and pathological reports). Primary outcomes were incidence of complications, intra-abdominal abscess and wound infection. Secondary outcomes were length of operation, length of hospital stay, resumption of diet, incidence of bowel obstruction, duration of antibiotic use and readmission. Laparoscopy appendectomy was performed with two working ports. Results: LA was performed in 123 children aged 2 to 14 years (mean of eight years) over a 3-year period, of whom only 34 cases had complicated appendicitis (either localized or generalized peritonitis). There was one conversion to open appendectomy (OA) in a patient with appendicular abscess with a mass, which excluded from our analysis. There were 6 patients with generalized peritonitis and 26 patients with localized abscess, and two patients with appendicular mass. The Average duration of symptoms was four days (ranged 3-6 days). The mean length of operation was 52 minutes (ranged 40-80 minutes). The average length of hospital stay was 4.4 days (ranged 4-7 days). They were able to restart oral intake from 16 to 48 hours postoperatively. Two patients (5%) had postoperative complications; one patient with intra-abdominal abscess who underwent reoperation and the second patient with umbilical wound infection was resolved with antibiotherapy. The average follow-up was 14 months (ranged from 4-36 months). Conclusions: We recommend laparoscopic approach for all children presenting complicated appendicitis as the initial procedure of choice.

Summary Background: Limited duration of analgesia is among the limitations of single caudal injection with local anesthetics. Therefore, the purpose of this study was to evaluate the effectiveness and safety of bupivacaine in combination with either neostigmine or tramadol for caudal block in children undergoing inguinal herniorrhaphy. Methods: In a double-blinded randomized trial, sixty children undergoing inguinal herniorrhaphy were enrolled to receive a caudal block with either 0.25% bupivacaine (1 ml·kg−1) with neostigmine (2 μg·kg−1) (group BN) or tramadol (1 mg·kg−1) (group BT). Hemodynamic variables, pain and sedation scores, additional analgesic requirements, and side effects were compared between two groups. Results: Duration of analgesia was longer in group BT (17.30 ± 8.24 h) compared with group BN (13.98 ± 10.03 h) (P = 0.03). Total consumption of rescue analgesic was significantly lower in group BT compared with group BN (P = 0.04). There were no significant differences in heart rate, mean arterial pressure, and oxygen saturation between groups. Adverse effects excluding the vomiting were not observed in any patients. Conclusion: In conclusion, tramadol (1 mg·kg−1) compared with neostigmine (2 μg·kg−1) might provide both prolonged duration of analgesia and extended time to first analgesic in caudal block.

BACKGROUND: Testicular torsion (TT), or twisting of the testicle resulting in a strangulation of the blood supply, occurs in men whose tissue surrounding the testicle is not well attached to the scrotum. It is important to emphasize that testicular torsion is a medical emergency. OBJECTIVES: The aim of this study is to evaluate the second look exploration and outcomes in TT. PATIENTS AND METHODS: Seventy boys out of 124 patients underwent early exploration and 48 hours later second look exploration due to TT. All patients were checked with preoperative color-doppler ultrasonography (CDU) and intraoperative bleeding test. Data included age at admission, side of pathology, relation of TT with season of year, duration of preoperative history, degree of testicular torsion, CDU findings, and degree of bleeding; results of second look exploration, follow-up, and outcomes were analyzed. RESULTS: Totally 70 patients were included in this study within five years, of which mean age was 28.6 ± 32.9 months (range 1 to 144), 48% of our patients had nausea and vomiting. Preoperative CDU showed absent/weak flow in 50 (71%) cases. Winter showed most frequently (44%) referred cases of testicular torsion. Orchidopexy was performed in 44 (63%) and orchidectomy in 26 (37%) cases after second look exploration. Mean follow-up duration was 3.1 ± 1.4 years. 4 (9%) cases in orchidopexy group developed testicular atrophy during follow-up, all four cases had a history of longer than 12 hours and grade II testicular bleeding test intra-operatively. Other orchidopexy patients salvaged. 26 patients, who were in grade III, underwent orchidectomy in second look exploration. CONCLUSIONS: TT requires emergency attention. The ischemia time of the testis is traditionally after 6 hours, and imaging or other diagnostic modality should not be a cause of delay. Early surgical exploration is modality of choice, and second look exploration after 48 hours can be more effective and salvageable in these patients.

Highlights • We report a newborn with soft palatal mass. • We excised the mass. • On the gross pathological examination, it was polypoid lesion covered with skin. Histologically, it consisted of a core of lobules of mature adipose tissue admixed with fascicles of striated skeletal muscle and covered by keratinized squamous epithelium containing many hair follicles associated with sebaceous and eccrine glands. • These findings confirmed mature teratoma., Teratoma is a true neoplasm that consists of tissues from all 3 embryonic germ layers: ectoderm, mesoderm, and endoderm. Nasopharyngeal teratoma is very rare. We present one case of nasopharyngeal teratoma attached to the soft palatal wall in a newborn.

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.

Objective: A neurogenic bladder is one which functions abnormally due to disorders of sacral nerves that control the bladder's ability to fill, store and empty urine. Abnormal bladder function can cause the bladder to be underactive or overactive. This study was planned to evaluate the treatment outcome of our patients with neurogenic bladder dysfunction (NGBD). Methods: Thirty three patients who have been treated for NGBD were evaluated. Diagnosis was confirmed by voiding-cysto-urethrography (VCUG) and urodynamic study. The patients were treated medically and all had clean intermittent catheterization (CIC). Data regarding age, sex, clinical and paraclinical findings, sonography, imagings, renal scan, associated anomalies, treatment and outcomes were collected and entered in SPSS software version18 and analyzed by descriptive statistical. Findings : Totally 33 patients aged three days to four years (mean 6.8 months) were included in this study. There were 20 (61%) males and 13 (39%) females. Mean follow-up period was 3.4±1.2 years (1.5 months to 5 years). Eighty two precent cases had bilatral and 18% unilatral hydronephrosis and bilatral vesicouretral reflux (VUR) existed in 67% and unilatral in 33% of the patients. Treatment consisted of antibiotherapy and CIC in all patients, which was only in 33% of the cases succesful. The most common associated anomaly was meningomyelocle in 8 patients. Vesicostomy was performed in 22 (67%) cases. Kidney scan showed scar in 10 patients at follow-up study. Complete continence on follow-up was achieved in 24 (71% ) patients, and it was improved in 6 (18% ) cases. Mortality rate was 9% (3 cases). Cure rate was 85% in urinary tract infection, 82.7% in hydronephrosis, 80% in VUR and 86.5% in kidney function. Conclusion: Anticholinergic medications was not effective in all our patients. We believe that permanent vesicostomy is an effective and acceptable surgical intervention for protection of upper urinary tract decompression, especially in those who do not respond to medical treatment and have high risk position.

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.

Objective Posterior urethral valves (PUV) are the most common cause of bladder outlet obstruction in infancy that impair renal and bladder function. This study was planned to evaluate and record the various clinical presentations and management, complications, and surgical management and long-term outcome of PUV. Methods In a retrospective study, 98 patients who have been treated for PUV are evaluated in Mofid Children's Hospital from January 2007 to December 2012. Detailed history taken and paraclinical examinations were performed in each patient and diagnosis was confirmed by voiding-cysto-urethrography (VCUG). PUV had been ablated in 62 patients by electric hook, and diversion was performed in 42 (42.85%) cases. Data were analyzed by SPSS software version18. Findings Totally 98 patients with mean age at diagnosis 62 (±13) days were included in this study. Fifty seven cases had been catheterized within one to 6 days of life (mean age one day), PUV was ablated in 62 patients by electric hook, and diversion was performed in 42 cases. The most common symptom in our group was dribbling poor stream 51% and urinary tract infection (UTI) 40.8%. There was vesico-ureteral-reflux (VUR) in 61.2%, and hydronephrosis in 82.6%. Most common associated anomaly was kidney anomalies (multicystic kidney disease and renal agenesis/dysplasia) in 8 (8.2%) patients. Twenty patients had prenatal diagnosis of PUV. Complication occurred in three (3.1%) patients. Mortality occurred in 5 (5.1%) patients. Mean follow-up period was 3.4±1.2 years (1.5 months to 5 years). Conclusion Urinary drainage by feeding tube in early days of infancy, followed by valve ablation is the best treatment in PUV, and urinary diversion improves the outcome. VCUG is still the gold-standard imaging modality for documenting PUVs. The factors like renal dysplasia and UTI have their role in final outcome.

Objective Muscle biopsy is a very important diagnostic test in the investigation of a child with suspected neuromuscular disorder. The goal of this study was to review and evaluate pediatric muscle biopsies during a 2-year period with focus on histopathology diagnosis and correlations with other paraclinic studies. Materials & Methods We investigated 100 muscle biopsies belonging to patients with clinical impression of neuromuscular disorder. These patients have been visited consecutively by pediatric neurologists during 2010 to 2012. Samples were investigated by standard enzyme histochemical and immunohistochemical techniques. Result Sixty-nine (69%) males and 39 (39%) females with a mean age of 5.7 years were evaluated. Major pathologic diagnoses were Muscular dystrophy (48 cases), Neurogenic atrophy (18 cases), nonspecific myopathic atrophy (12cases), congenital myopathy (6 cases), storage myopathies (4 cases) and in 6 cases there was no specific histochemical pathologic finding. EMG was abnormal in 79 cases. Degree of correlation between EMG and biopsy result was significant in children ≥ 2 years of age. Conclusion This study confirms the high diagnostic yields of muscle biopsy especially only if standard and new techniques such as enzyme study and immunohistochemistry are implemented. Also, we report 11 cases of Merosin negative congenital muscular dystrophy. This is the largest documented case series of Merosin deficient congenital muscular dystrophy reported from Iran.

Background Acute mediastinitis is a serious medical condition with a mortality rate of 30 to 40% or even higher. Early diagnosis with prompt and aggressive treatment is essential to prevent its rapid progression. We evaluated acute mediastinitis cases and analyzed the outcomes. Materials and Methods A retrospective chart review was conducted on patients diagnosed with acute mediastinitis who were admitted to Mofid Children's Hospital from January 2001 to January 2010. Results Seventeen patients aged 1 to 10 yrs. (mean =3.8 yrs) were evaluated including 12 (70%) boys and 5 (30%) girls. The most common symptoms were fever, dyspnea, cyanosis, tachycardia and tachypnea. The etiology of mediastinitis was iatrogenic esophageal perforation (EP), and related to manipulation in 13(77%), and leakage of esophageal anastomosis in 4 cases (33%). The underlying diseases were esophageal atresia in 2(12%), corrosive injury of the esophagus in 13(76%), congenital esophageal stenosis in one (6%), and gastroesophageal reflux esophagitis also in one (6%) patient. Patients with clinical symptoms were evaluated by immediate chest radiography, and gastrografin swallow. After early diagnosis, the patients received wide spectrum antibiotics and immediate mediastinal or thoracic drainage, followed by esophagostomy and gastrostomy. Only one case of endoscopic perforation was managed by NG tube. Fifteen patients (88%) survived successfully. We had 2(12%) cases of mortality in our study (one patient after esophageal substitution, mediastinal abscess and septicemia, and the other one developed esophageal perforation 6 months after early management and died of cardiac arrest during endoscopic dilation). Conclusion Prevention of acute mediastinitis is still a difficult challenge. As the prognosis is not good and patients have high mortality, rapid management is mandatory.

Saeid Aslanabadi1, Kamyar Ghabili2, Mohsen Rouzrokh3, Mohammad Bagher Hosseini1, Masoud Jamshidi1, Farzad Hami Adl4, Mohammadali M Shoja51Pediatric Health Research Center, Tabriz University of Medical Sciences, Tabriz; 2Tuberculosis and Lung Disease Research Center, Tabriz University of Medical Sciences, Tabriz; 3Department of Pediatric Surgery, Mofid Hospital, Shaheed Beheshti University of Medical Sciences, Tehran; 4Faculty of Medicine, Tabriz University of Medical Sciences, Tabriz; 5Tuberculosis and Lung Disease Research Center, Tabriz University of Medical Sciences, Tabriz, IranBackground: Predicting the presence of long-gap esophageal atresia (EA) prior to the surgery is of clinical importance. No comparison between short-gap and long-gap EA for the prevalence of VACTERL and non-VACTERL-type anomalies has yet been performed.Objective: The aim of this study was to compare VACTERL and non-VACTERL-type anomalies between patients with short-gap and long-gap EA.Methods: Retrospectively, medical records of all newborns managed for EA/tracheoesophageal fistula (TEF) in Tabriz Children's Hospital and Tehran Mofid Hospital between 2007 and 2010 were evaluated. Demographic data and associated anomalies including both the VACTERL and non-VACTERL-type defects were listed. The VACTERL spectrum defects covered vertebral/costal, anorectal, cardiovascular, TEF, and renal- or radial-type limb anomalies. The non-VACTERL-type anomalies included hydrocephalus, orofacial defects, respiratory system anomalies, gastrointestinal anomalies, genital anomalies, and non-VACTERL limb defects. Demographic data, and the VACTERL and non-VACTERL-type anomalies were compared among children with long-gap EA and those with short-gap EA.Results: Two hundred and seventy-six children were included in the study: 230 (83.3%) in the short-gap EA group and 46 (16.7%) in the long-gap EA group. Although prevalence of the VACTERL spectrum anomalies did not differ between the two groups, the non-VACTERL anomaly was more common in the long-gap EA group (P = 0.02). Among the VACTERL-type defects, TEF was detected in 30 (65.2%) and 218 (94.7%) patients in long-gap and short-gap EA groups, respectively (P = 0.0001).Conclusion: The non-VACTERL-type anomalies, but not the VACTERL spectrum defects, are more frequent in patients with long-gap EA than those with short-gap EA.Keywords: esophageal atresia, short-gap, long-gap, tracheoesophageal fistula, anomaly, VACTERL

Limited duration of analgesia is among the limitations of single caudal injection with local anesthetics. Therefore, the purpose of this study was to evaluate the effectiveness and safety of bupivacaine in combination with either neostigmine or tramadol for caudal block in children undergoing inguinal herniorrhaphy.In a double-blinded randomized trial, sixty children undergoing inguinal herniorrhaphy were enrolled to receive a caudal block with either 0.25% bupivacaine (1 ml x kg(-1)) with neostigmine (2 microg x kg(-1)) (group BN) or tramadol (1 mg x kg(-1)) (group BT). Hemodynamic variables, pain and sedation scores, additional analgesic requirements, and side effects were compared between two groups.Duration of analgesia was longer in group BT (17.30 +/- 8.24 h) compared with group BN (13.98 +/- 10.03 h) (P = 0.03). Total consumption of rescue analgesic was significantly lower in group BT compared with group BN (P = 0.04). There were no significant differences in heart rate, mean arterial pressure, and oxygen saturation between groups. Adverse effects excluding the vomiting were not observed in any patients.In conclusion, tramadol (1 mg x kg(-1)) compared with neostigmine (2 microg x kg(-1)) might provide both prolonged duration of analgesia and extended time to first analgesic in caudal block.

Hirschsprung's disease (HD) is a relatively common congenital disease that could be suspected by clinical symptoms, abdominal plain X-ray, and finally diagnosed by rectal biopsy. In 80% cases, rectosigmoid junction is involved. Recently, one-stage transanal pull-through (TAPT) procedure has been popular and may have several complications.During a 4-year period, 86 infants (28 girls, 58 boys) with mean age 8 days (3-33) and clinically suspected to HD were admitted in our center. HD was proved by rectal biopsy. All patients after full bowel preparation and rectal washout were candidates for TAPT operation. A Swenson-like procedure was performed and the anastomosis was done between the well blood supply ganglionic colon and the rectum at 1 cm above dentate line. Interrupted suture with 5-0 Vicryl was used. Nelaton tube (12 F) inserted in the pelvis via transprineal for drainage of blood or collection. From February 2008 in 30 cases, prophylactic Hegar dilatation was performed 2 weeks after operation.Anal stricture in 12 cases (14%) was treated by anal dilation in 10 cases and 2 cases corrected by surgical management. Entrocolitis in 4 cases (5%) was treated by medical management. In two cases, retrocolic abscess had spontaneous drainage via tube drain. There was no anastomotic stricture after starting prophylactic anal bouginage.TAPT has many advantages, low complications and the results are excellent. It seems the most common complication is anastomotic stricture that responds well to prophylactic bouginage. We recommend prophylactic anal bouginage with Hegar probe at 2 weeks after operation. Long-term follow-up is needed to evaluate the outcomes of our operations.

Histological typing, especially the evidence of anaplasia and stage of the tumor are two major prognostic indicators in Wilms tumor, but some genetic factors have recently been noted. Mutations in TP53, which is a tumor suppressor gene, have been demonstrated to be associated with poor prognosis in some malignancies. There are also few studies which have confirmed the correlation between p53 protein overexpression and advanced course in Wilms tumor. This study was conducted to determine the correlation p53 immunoexpression and prognosis and outcome of Wilms tumor.The overexpression of p53 in 44 specimens of children (26 boys and 18 girls) with Wilms tumor (median age, 36 months; range, 4 to 96 months) was assessed in Mofid Children's Hospital, Tehran, Iran. The overexpression of p53 was determined by immunohistochemistry with antihuman p53 antibody.A positive immunostaining for p53 was detected in 24 of the 44 cases (54.7%). The grade of the tumor and anaplasia were associated with p53 positivity, but there was no association of p53 with the tumor stage. In comparison to p53 negatives, week-moderate and high p53-expressing tumors had significantly decreased the overall survival (hazard ratio, 3.75 and 8.61; P = .05 and P = .01, respectively).Overexpression of p53 protein in Wilms tumor is an indicator of poor pro gnosis, because it is correlated with unfavorable histology tumors and a shorter survival period.

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.

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.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).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%).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.

Congenital cutis laxa is a genetically heterogeneous condition presenting in the newborn with loose, redundant skin folds, decreased elasticity of the skin, and general connective tissue involvement. A 2-day-old full term neonate with congenital cutis laxa presented with respiratory distress. Investigations revealed huge hiatal hernia. Patient underwent loose Nissen’s fundoplication. In postoperative period patient developed intussusception which was manually reduced at re-surgery.