Aim of the Review: To describe the significant improvement in the diagnosis, treatment and outcome of children diagnosed with hepatoblastoma (HB) that has occurred in the past four decades. Recent findings are mainly focused on lessons learned from the experiences of the Childhood Liver Tumors Strategy Group (SIOPEL). Important milestones were the risk stratification of HB that allowed to tailor down therapy for standard-risk HB and intensify treatment for high-risk HB. The multi-institutional international cooperative SIOPEL trials are reviewed and current treatment guidelines are given. Intensified cooperation between the SIOPEL and the Children's Oncology Group (COG) and the national study groups from Germany (GPOH) and Japan (JPLT) led to the acceptance and use of one staging system (PRETEXT) and the formation of a single robust database containing data of 1605 HB patients. This will allow analysis with enough statistical power of treatment directing factors that will form one of the bases of the next-generation clinical trial that is currently designed by all four collaborating study groups. Summary: Successive SIOPEL trials and increasing international collaboration have improved survival rates of patients with HB through risk stratification, advances in chemotherapy and increased complete resection rates including liver transplantation as a surgical option.

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With the availability of several protocols in the management of Wilms' tumor, there is dilemma in the minds of the treating oncologists or pediatric onco-surgeons as to whether the child should receive upfront chemotherapy or should be operated upon primarily. It is necessary for us to understand why do we follow either of the protocols, NWTS which follows the upfront surgery principle or the SIOP which follows the upfront chemotherapy principle in all stages of the disease. While deciding which protocol to follow, it is imperative to know the pros and cons of the treatment strategies and also to study the outcome patterns in both the treatment regimes which is what this article highlights. In an attempt to compare all the differences in both the major protocols, it was realized that most of our patients in the Indian scenario present with advanced disease and thus poorer outcomes if intensive and appropriate treatment strategies are not utilized. Hence, it is imperative that we should study our own patients through the Indian Wilms' tumor study group and adopt the policies which improve the overall event free survival on a nationwide basis.

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Vocational and social issues affect rehabilitation and development of patients with cleft lip and cleft palate. However, psychological problems like lowered self esteem and difficulties in social interaction have also been noted in them. Not many pediatric reconstructive surgery teams have a psychiatrist on their panel. It is likely that psychological problems are higher in incidence than literature actually suggests. Hence it is very essential that such cases are identified by the surgical team to maximize positive outcome of surgery and rehabilitation. This study discusses psychological issues revolving around cleft lip and cleft palate along with lacunae in many psychological research studies.

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The medicinal properties of honey have been recognized since antiquity. Although used as an adjuvant method of accelerating wound healing from ancient times, honey has been sporadically used in the treatment of burns. Honey acts mainly as a hyperosmolar medium and prevents bacterial growth. Because of its high viscosity, it forms a physical barrier, and the presence of enzyme catalase gives honey an antioxidant property. Its high-nutrient content improves substrate supply in local environment promoting epithelialization and angiogenesis. In pediatric burn patients no exclusive study has been conducted using honey as a burn dressing. An attempt is being made to evaluate the effect of honey in the management of burns in pediatric patients.

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Congenital pouch colon (CPC), a condition associated with anorectal agenesis has been reported mainly from the Indian subcontinent though there have been few case reports from other areas. The pouch differs from a normal colon structurally, histologically and functionally. The management involves a diversion colostomy at birth with or without the excision of pouch followed by pull through. This review article attempts to compile all the available literature on pouch colon along with the authors' experience and discuss the relevant issues for proper diagnosis and management. A changing trend has been seen in the most common type of CPC seen over the years from complete congenital pouch colon that accounted for more than 70% of cases earlier to incomplete pouch colon that is more commonly seen now.

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Anorectal malformations (ARMs) are among the more frequent congenital anomalies encountered in paediatric surgery, with an estimated incidence ranging between 1 in 2000 and 1 in 5000 live births. Antenatal diagnosis of an isolated ARM is rare. Most cases are diagnosed in the early neonatal period. There is a wide spectrum of presentation ranging from low anomalies with perineal fistula having simple management to high anomalies with complex management. Advances in the imaging techniques with improvement in knowledge of the embryology, anatomy and physiology of ARM cases have refined diagnosis and initial management. There has been marked improvement in survival of such patient over the last century. The management of ARM has moved forward from classical procedures to PSARP to minimal invasive procedures. But still the fecal and urinary incontinence can occur even with an excellent anatomic repair, mainly due to associated problems. There has been a paradigm shift in approach to these patients which involves holistic approach to the syndrome of Anorectal malformations with a long term goal of achievement of complete fecal and urinary continence with excellent quality of life.

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We report two cases of gastrointestinal perforation by ventriculoperitoneal (VP) shunts and review the literature on the topic. The time interval between shunt surgery and detection of bowel perforation is minimum in infants and increases with age. Sigmoid and transverse colon followed by stomach are the most frequent sites of gastrointestinal perforations by VP shunts.

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Aim: To assess the various epidemiological parameters that influence the causation of trauma as well as the consequent morbidity and mortality in the pediatric age group. Materials and Methods: A prospective study of 791 patients of less than 12 years age, was carried out over a period of 1 year (August 2009 to July 2010), and pediatric trauma trends, with regards to the following parameters were assessed: Age group, sex, mode of trauma, type of injury, place where the trauma occurred and the overall mortality as well as mortality. Results: Overall trauma was most common in the school-going age group (6-12 years), with male children outnumbering females in the ratio of 1.9:1. It was observed that orthopedic injuries were the most frequent (37.8%) type of injuries, whereas fall from height (39.4%), road traffic accident (27.8%) and burns (15.2%) were the next most common modes of trauma. Home was found out to be the place where maximum trauma occurred (51.8%). Maximum injuries happened unintentionally (98.4%). Overall mortality was found out to be 6.4% (n = 51). Conclusions: By knowing the epidemiology of pediatric trauma, we conclude that majority of pediatric injuries are preventable and pediatric epidemiological trends differ from those in adults. Therefore, preventive strategies should be made in pediatric patients on the basis of these epidemiological trends.

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Aim: To assess the epidemiology, pattern, and outcome of trauma in pediatric population. Materials and Methods: A total of 1148 pediatric patients below 15 years of age presenting in the emergency department of our hospital were studied over a period of 3 years. The patients were categorized into four age groups of <1 year, 1-5 years, 6-10 years, and 11-15 years. The data were compared regarding mode of trauma, type of injury, place of injury among different age groups and both sexes. Results: The majority of the pediatric trauma cases were seen in males 69.86%, (n = 802) and females comprised only 30.13% (n = 346). Road traffic accident (RTA) was the most common mode of trauma in male children, i.e. 59.47% (n = 477) followed by fall injuries, i.e. 29.42% (n = 236). In females, fall was the most common mode of trauma, i.e. 52.31% (n = 181) followed by RTA (36.70%, n = 127). Fall injuries occurred mostly at homes. Among RTA, hit by vehicle on road while playing was most common followed by passenger accidents on two wheelers, followed by hit by vehicle while walking to school. Among fall, fall while playing at home was the most common. Out of total 1148 patients, 304 (26.48%) comprised the polytrauma cases (involvement of more than two organ systems), followed by abdominal/pelvic trauma (20.99%, n = 241), followed by head/face trauma (19.86%, n = 228). Out of total 1148 patients admitted over a period of 36 months, 64 died (5.57%). 75 (6.5%) patients had some kind of residual deformity or disability. Conclusion: The high incidence of pediatric trauma on roads and falls indicate the need for more supervision during playing and identification of specific risk factors for these injuries in our setting. This study shows that these epidemiological parameters could be a useful tool to identify burden and research priorities for specific type of injuries. A comprehensive trauma registry in our set up seems to be important for formulating policies to reduce pediatric trauma burden.

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Aims and Objectives: Delayed management of anorectal malformation (ARM) increases the surgical and functional complications for the patient. We defined "delayed presentation of ARM" and reviewed our patients with ARM to find out the incidence and causes of delayed presentation. Materials and Methods: Patients satisfying the criteria of "delayed presentation of ARM" were involved. Detailed information of each patient including the mode of presentation, associated anomalies, plan of management and follow-up was obtained from the hospital records. Results: Between 2003 and 2006, 43 patients satisfied our criteria of "delayed presentation of ARM". There were 21 males and 22 females. Seventeen of these males presented with low-type ARM. Eleven of them were managed by a single-stage procedure. These "delayed presenters" had to live with constipation, inadequate weight gain and parental anxiety for a greater time. Analysis of the outcomes showed more functional complications in patients who had undergone failed perineal surgery previously. In females with low ARM, the procedure of choice was anterior sagittal anorectoplasty (ASARP). Single stage surgery provides good outcomes for most of low type of ARMs. High-type ARMs in males and females were managed by a staged procedure. Conclusion: "Delayed presentation of ARM" is a major group of ARM in our setup. The management and results of their treatment are not different from those of the early presenters. The most common cause of delayed ARM is wrong advice given by the health care providers followed by inadequate treatment elsewhere. Corrective surgeries taking second attempt in perineum always produces poor outcomes.

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Aim: Crossed fused renal ectopia is a rare congenital malformation, which is reported to be usually asymptomatic but may have varied presentations. This survey was conducted to study the clinical profile and the challenges posed in the management of this entity. Materials and Methods: Retrospective analysis of 6 patients diagnosed to have crossed fused renal ectopia during 1997-2010. The diagnosis was confirmed during surgical exploration in one patient. In one patient it was detected on antenatal ultrasonography and in the other 4 patients it was detected during investigations for abdominal pain, abdominal mass, anorectal malformation and urinary tract infection. Results: The left moiety was crossed and fused with the right moiety in 4 cases. Ultrasonography was found to be a good screening investigation with useful diagnostic contributions from CT scans, radionuclide scintigraphy and magnetic resonance urography. Micturating cystourethrography revealed presence of VUR in 4 cases, 3 of whom have undergone ureteric reimplantation. Two patients required pyeloplasty for pelviureteric junction obstruction; in one of these patients the upper ureter was entrapped in the isthmus. In one patient, a non-functioning moiety resulted in nephrectomy. All children were asymptomatic at last follow-up with stable renal functions. Conclusions: Crossed fused renal ectopia was detected in most patients during investigation for other problems. It was found more commonly in boys. The left moiety was crossed to the right in the majority of cases. Associated urological problems were found in most cases and required the appropriate surgical management.

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We report a neonate with acute appendicitis who was mistakenly diagnosed and treated initially as neonatal necrotizing enterocolitis. The diagnostic dilemma of this rare and life-threatening condition in premature babies and newborns is underlined. Awareness of this rare condition and possible differential diagnosis in this age group is also discussed.

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The need for successful management of posterior urethral valves always captivates the minds of pediatric surgeons. Its success, however, depends on several factors ranging from prenatal preservation of upper tracts to postoperative pharmacological compliance. Regardless of measures available, some cases do not respond and progress to end stage. The management depends on several issues ranging from age and severity at presentation to long-term follow-up and prevention of secondary renal damage and managing valve bladder syndrome. This article is based on a consensus to the set of questionnaires, prepared by research section of Indian Association of Paediatric Surgeons and discussed by experienced pediatric surgeons based in different institutions in the country. Standard operating procedures for conducting a voiding cystourethrogram and cystoscopy were formulated. Age-wise contrast dosage was calculated for ready reference. Current evidence from literature was also reviewed and included to complete the topic.

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Aim: To assess the incidence of different head and neck cancers in pediatric age group in a referral hospital. Methods: In this prospective study, children below the age of 12 years underwent a thorough clinical, ENT examination and the diagnosis was conformed histologically in all the cases. Results: Fifty-three were diagnosed to be suffering from different head and neck neoplasms among 21,216 children (0.25%). Male-to-female sex distribution was 1.78:1. The lymphomas were the most common (43.39%) followed by the rhabdomyosarcoma (20.75%) and the nasopharyngeal carcinoma (15.09%). Of the lymphomas, the non-Hodgkin's lymphoma was predominant (26.41%). Other lesions were thyroid carcinomas and mucoepidermoid carcinoma of parotid. Conclusions: Malignancy should always be considered in the differential diagnosis of masses in the head and neck region in children.

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Formulation of research question (RQ) is an essentiality before starting any research. It aims to explore an existing uncertainty in an area of concern and points to a need for deliberate investigation. It is, therefore, pertinent to formulate a good RQ. The present paper aims to discuss the process of formulation of RQ with stepwise approach. The characteristics of good RQ are expressed by acronym “FINERMAPS” expanded as feasible, interesting, novel, ethical, relevant, manageable, appropriate, potential value, publishability, and systematic. A RQ can address different formats depending on the aspect to be evaluated. Based on this, there can be different types of RQ such as based on the existence of the phenomenon, description and classification, composition, relationship, comparative, and causality. To develop a RQ, one needs to begin by identifying the subject of interest and then do preliminary research on that subject. The researcher then defines what still needs to be known in that particular subject and assesses the implied questions. After narrowing the focus and scope of the research subject, researcher frames a RQ and then evaluates it. Thus, conception to formulation of RQ is very systematic process and has to be performed meticulously as research guided by such question can have wider impact in the field of social and health research by leading to formulation of policies for the benefit of larger population.

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Pediatric germ cell tumors (GCT) are rare tumors: 80% are benign, 20% malignant (2-3% of all malignant pediatric tumors). The gonadal sites (ovary and testis) account for 40% of cases. Ovarian GCTs: Represent 30% of GCTs and 70% of neoplastic ovarian masses, being the most common ovarian neoplasms in children and teenagers. Benign and immature forms (teratomas) constitute about 80% of all ovarian GCTs, malignant forms represent 20% increasing during adolescence. The most common malignant entity in children is the yolk sac tumors (YST); dysgerminoma is frequent during adolescence and being bilateral in 10% of cases. Presentation is similar in malignant and benign lesions; abdominal pain (70-80%) and lower abdominal mass are common symptoms. Evaluation of alpha-fetoprotein (αFP) or beta subunit of human chorionic gonadotropin (βHCG) is essential to address the nature of the tumors: Their elevation means presence of malignancy. Surgery includes intraoperative staging procedures and requires ovariectomy or ovarosalpingectomy for malignant lesions, but may be conservative in selected benign tumors. Since malignant GCTs are very chemosensitive, primary chemotherapy is recommended in metastatic or locally advanced tumors. Testicular GCT: Represent 10% of pediatric GCT, and about 30% of malignant GCT with two age peaks: Children <3 years may experience mature teratoma and malignant GCTs, represented almost exclusively by YST, while adolescents may also show seminomas or other mixed tumors. The main clinical feature is a painless scrotal mass. Surgery represents the cornerstone of the management of testicular GCTs, with an inguinal approach and a primary high orchidectomy for malignant tumors, while a testis-sparing surgery can be considered for benign lesions. A retroperitoneal lymph node (LN) biopsy may be necessary to define the staging when the involvement of retroperitoneal LN is uncertain at imaging investigations. Conclusion: Patients with gonadal malignant GCTs fare better than those with extragonadal mediastinal germ cell tumors (MGCTs) and survival rate exceeds 90% in localized forms. Chemotherapy has significantly improved the outcome of malignant forms since the introduction of platinum based regimens. The surgical procedure has to be performed in agreement with the ongoing protocols.

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Aim: Objective of this study is to report our experience with sigmoid vaginoplasty in adolescents. Materials and Methods: A retrospective study of children with vaginal atresia and Mayer-Rokitansky-Kuster-Hauser syndrome. The sigmoid segment was used for vaginoplasty in all the cases. Results: Eight children were studied over a period of 7 years. The postoperative complications were ileus in 2, mucosal prolapse of the neovagina in 1, and minor wound infection in 1 patient. Seven patients are on regular follow-up. All the neovaginas were patent and functional. One patient had unacceptable perineal appearance, that is, badly scarred perineum as a late complication. None of the patients had vaginal stenosis or excessive mucus discharge, during follow-up visits. Out of the 7 patients, 2 patients are sexually active and satisfied. Conclusions: Sigmoid vaginoplasty is a safe and acceptable procedure for vaginal agenesis with good cosmetic results and acceptable complications rate. Sigmoid colon vaginoplasty is the treatment of choice because of its large lumen, thick walls resistant to trauma, adequate secretion allowing lubrication, not necessitating prolonged dilatation, and short recovery time.

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Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is relatively rare but one of the most important causes of severe neonatal hypoglycemia. Recognition of this entity becomes important due to the fact that the hypoglycemia is so severe and frequent that it may lead to severe neurological damage in the infant manifesting as mental or psychomotor retardation or even a life-threatening event if not recognized and treated effectively in time. Near-total pancreatectomy may be required for patients with intractable hypoglycemia despite medical treatment; however, that may result in diabetes mellitus or recurrent postoperative hypoglycemia. This review aims to consolidate the traditional concepts and current information related to the pathogenesis and management of PHHI.

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Splenic cysts are rare lesions. Primary cysts have a cellular lining that can be caused by congenital events or parasitic infection (Echinococcus). Secondary cysts have no cellular lining and may be of hemorrhagic, serous, inflammatory, or degenerative origin. We report a case of pseudocyst treated successfully by splenectomy, and we review the literature.

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The pathology of salmonellosis after a faeco-oral transmission was first clearly described by Jenner in 1850. Over the years, the pathological manifestations in different tissues of the body have been described. The ileum is however mostly involved leading to enlarged Peyer's patches, ulceration, and sometimes bleeding and perforation. Efforts at control have largely been improvement in public water supply, safe disposal of waste, and general public health measures. Despite these measures, intestinal perforation from salmonellosis remains the commonest cause of emergency operation in children above 3 years. The incidence continues to rise, so also the mortality, despite new antibiotics and improvement in facilities in the hospitals. Even more disturbing is that we now see more perforations per patient, and more involvement of the colon. Three recently managed patients with multiple ileal/colonic perforations were reviewed. Presenting problems, delay in referral, choice of antibiotics and postoperative complications were noted. One patient had 27 perforations and another 14 perforations. Both survived. Is salmonella changing? Are our patients changing? Is the environment changing? Are the goal posts changing? This article details our recent experience with this dreadful disease, reviews the new literature and makes suggestions for the way forwards.

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Aims and Objective: To postulate a hypothesis to explain the embryogenesis of exstrophy bladder based on our clinical observations. Materials and Methods: In 27 cases of exstrophy, we measured the distance between the lowermost inguinal skin crease to the root of the penis (clitoris) (B) and the distance between the penis (clitoris) and the scrotum (labia majora) (C). These were compared with age, height and XP distance (distance between xiphisternum and symphysis pubis) matched control group of normal children. The distance between the lowermost inguinal skin crease and the penis (clitoris) (A) was measured in control group. Results: The observation was A = B + C. This implies that in exstrophy bladder, the position of the penis (clitoris) has moved cephalad from the lower border of A to the junction of B and C. Conclusion: Based on the observations, we postulate that abnormal origin of genital tubercle may be the cause of exstrophy bladder. The abnormal origin of primordia of the genital tubercle in more cephalad direction than normal causes wedge effect, which will interfere with the medial migration of the mesoderm as well as the midline approximation of mesodermal structures in the lower abdominal wall, thereby resulting in the exstrophy of bladder.

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Aim: To introduce a new classification for the congenital anomaly of webbed penis and suggest an operative technique that can be planned according to the severity of webbing. Materials and Methods: A prospective study was conducted in two pediatric surgical units in Egypt and UAE on babies who were referred for circumcision. A preplanned written protocol was designed before commencing the study. Results: A total of 5,881 babies aged from 1 day to 6 months were seen in two pediatric surgical units. The webbed penis is broadly classified into primary and secondary types. The primary is further subdivided into simple and compound. Conclusion : We believe that the new classification will serve as a baseline for the anatomical variants and help to streamline the operative procedure accordingly.

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Empyema thoracis can produce significant morbidity in children if inadequately treated. Correct evaluation of the stage of the disease, the clinical condition of the child and proper assessment of the response to conservative treatment is crucial in deciding the mode of further surgical intervention. This ranges from intercostal chest tube drainage and video-assisted thoracoscopic surgery to open decortication. Surgical decortication becomes mandatory in neglected cases; it gives very gratifying results ameliorating the disease rapidly and is well tolerated by young patients. This article reviews the current literature and discusses the important considerations while managing these patients. Indications for surgery are highlighted, based on our large experience at a tertiary care center.

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Tumors arising from the soft tissues are uncommon in children, accounting for about 6% of all childhood malignancies. More than half (53%) of these originate from the striated muscles and are called rhabdomyosarcomas (RMS) the remaining are nonrhabdomyosarcoma soft tissue sarcomas (NRSTS). Almost two-thirds of RMS cases are diagnosed in children <6 years of age. They can arise at varied locations like the head and neck region, genitourinary tract, extremities, trunk and retroperitoneum. Pathologically RMS is now classified as superior, intermediate and poor outcome histologies. For stratification of treatment and also comparison of results the RMS are now staged both by the clinical grouping and the TNM staging systems. The ultimate outcome depends on the site, extent of disease and histology. Currently, approximately 70% of the patients survive for 5 years or more and are probably cured. This is credited to the use of multi-modal, risk-adapted therapy, refinements in tumor grouping and better supportive care which has emerged out of cooperative studies like Intergroup Rhabdomyosarcoma Study (IRS) and the International Society of Pediatric Oncology studies (SIOP). The treatment involves chemotherapy, radiotherapy and organ/function preserving surgery. The gold standard chemotherapy is still vincristine, actinomycin D and cyclophosphamide (VAC) regime with high doses of intensity bone marrow rescue with colony stimulating factors. The NRSTS are rare and of heterogenous histologies and so it has been difficult to arrive at a treatment strategy for these. What is definitely understood is that these are usually immature and poorly differentiated tumors that respond poorly to chemotherapy and so surgical resection forms the mainstay of treatment with adjuvant radiotherapy and chemotherapy to prevent local recurrences. In all likelihood, the molecular analysis of RMS will further refine current classification schemes and knowledge of genetic features of the tumors will significantly improve the ability of investigators to identify patients at lower or higher risk of treatment failures, thus paving the way for advances in risk-based therapy.

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This review article covers the spectrum of congenital thoracic wall deformities along with their historical background. Willital's classification divides these deformities into 11 types - funnel chest (4 types), pigeon chest (4 types), and combination of funnel and pigeon chest, chest wall aplasia and cleft sternum. Records of patients at our center comprised 90% depression deformities, 6% protrusion deformities, 3% combined depression-protrusion deformities and 1% other forms. Mild forms of abnormalities warrant the wait- and-watch approach during the first 4-5 years. The deformities manifest primarily during the pubertal spurt often with rapid progression with subjective complaints like dyspnea, cardiac dysthesia, limited work performance and secondary changes. Operative correction in young adults is more favorable in mild cases. The Willital technique has been the standard technique for the correction of pectus excavatum, pectus carinatum and other combined forms of deformities at our center with excellent long term results. The Nuss procedure and the Pectus Less Invasive Extrapleural Repair (PLIER) technique for pectus excavatum and pectus carinatum have also been described in this article. Surgical correction for Poland's syndrome is reserved for patients with severe aplasia of the ribs with major depression deformity. Sternal defects including various types of ectopia cordis are discussed. Even after surgical correction, there is significant reduction in the total capacity and inspiratory vital capacity of the lungs, probably a result of the decreased compliance of the chest wall. However, the efficiency of breathing at maximal exercise improves significantly after operation.

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