A rare case of thoracoschisis, diaphragmatic and supraumbilical abdominal wall defect with right upper limb agenesis in a neonate is reported.

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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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Clitoromegaly in pediatric and adolescent age group is usually indicative of a disorder of sexual differentiation. We report a girl child presenting with clitoral enlargement due to an epidermoid cyst. The cyst was excised with complete cosmetic recovery.

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ABSTRACT: Duplication of the penis is an extremely rare anomaly. Approximately 100 cases have been reported since the first case report by Wecker in 1609. There are broadly three types of diphallus,viz. true diphallus with two independent penises, bifid phallus that may be glandular or complete and pseudodiphallus having a rudimentary phallus in addition to the normal penis. Numerous associated genitourinary and gastrointestinal anomalies have been described with diphallus. We encountered a complete bifid phallus in a 2 year old boy, with epispadias and a yentral preputial hoo, making the diphallic organ completely buried within it. In our exhaustive review of the literature, we did not come across any other case of this variety of the penile duplication that we termed the 'concealed or buried diphallus'. We did the corrective surgery in one stage with a good result.

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In a child who is toilet trained the sudden onset of daytime wetting with frequency or urgency is alarming to the parents. Initially this subject was subdivided into a number of descriptive clinical conditions which led to a lot of confusion in recognition and management. Subsequently, the term elimination dysfunction was coined by Stephen Koff to emphasise the association between recurrent urinary infection, wetting, constipation and bladder overactivity. From a urodynamic point of view, in voiding dysfunction, there is either detrusor overactivity during bladder filling or dyssynergic action between the detrusor and the external sphincter during voiding. Identifying a given condition as a 'filling phase dysfunction' or 'voiding phase dysfunction' helps to provide appropriate therapy. Objective clinical criteria should be used to define voiding dysfunction. These include bladder wall thickening, large capacity bladder and infrequent voiding, bladder trabeculation and spinning top deformity of the urethra and a clinically demonstrated Vincent's curtsy. The recognition and treatment of constipation is central to the adequate treatment of voiding dysfunction. Transcutaneous electric nerve stimuation for the treatment of detrusor overactivity, biofeedback with uroflow EMG to correct dyssynergic voiding, and behavioral therapy all serve to correct voiding dysfunction in its early stages. In established neurogenic bladder disease the use of Botulinum Toxin A injections into the detrusor or the external sphincter may help in restoring continence especially in those refractory to drug therapy. However in those children in whom the upper tracts are threatened, augmentation of the bladder may still be needed.

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OBJECTIVE: The aim of the study was to assess preputial retractability in children at various ages. MATERIALS AND METHODS: Nine hundred and sixty boys attending the hospital were included in the study. Children with hypospadias or history of preputial manipulation were excluded. Preputial anatomy was studied and subjects were classified into five groups as described by Kayaba et al . RESULTS: The prepuce could not be retracted at all so as to make even the external urethral meatus visible in 61.4% children aged 0-6 months while this decreased to only 0.9% in children aged 10-12 years. At the other end of the spectrum, while prepuce could not be fully retracted in any child below 6 months, it could be done in about 60% in the age group of 10-12 years. CONCLUSION Preputial nonseparation is the major cause of preputial nonretraction in the pediatric age group. Prepuce spontaneously separates from the glans as age increases and true phimosis is rare in children. Surgical intervention should be avoided for nonseparation of prepuce.

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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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Wilms' tumor (WT) represents 6% of childhood cancers. Recent advances in molecular biology have significant implications for the clinical management. A dramatic improvement in overall cure has resulted from the adoption of multimodality treatment during the past few decades. National Wilms' Tumor Study (NWTS), and the Sociιtι Internationale d'Oncologie Pιdiatrique (SIOP) have laid down the guidelines for standardized treatment of WT, though differing in the diagnostic and therapeutic approach. Both these groups currently aim at intensifying treatment for patients with poor prognosticators while appropriating the therapy to reduce long-term complications for those with favorable prognostic features. Challenges faced in developing nations include poverty, malnutrition, ignorance, and presentation in advanced stages coupled with limited facilities that are necessary for total management of these cases. In this article, we have discussed our approach to deal with patients with nephroblastoma, reviewed the literature on the current management strategies and the long-term outcome. Most countries have adopted the NWTS protocols, while others especially in Europe, South America and some Asian countries follow the SIOP regimen. Both have their advantages and weaknesses and maynot necessarily be suitable to the setup in developing countries. We have discussed the controversial issues in the management of WT including the timing of biopsy, type of biopsy, investigative approach, role of chemotherapy / radiotherapy, management of bilateral Wilms' tumor and parenchymal sparing renal surgery. Despite deviating from NWTS at various points, the overall results have remained satisfactory. Thus, developing countries might adopt their own protocols depending on the prevalent situations and facilities available to them to treat such patients.

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A stem cell is an undifferentiated cell in the body with undetermined function capable of forming various tissues under definite signals received from the body. Stem cell research in animals using embryonal stem cells has been an ongoing program in the west with fruitful results. However, only limited information is available with the use of stem cells in human beings. Of the various sources of stem cells, umbilical cord blood stem cell research has shown potential for future treatment in Alzheimer's, Parkinson's, heart attack, stroke and spinal cord injuries. Human trials have been done in diseases like spinal cord injury and chronic liver cirrhosis. Cord blood stem cells have already been effectively used in the treatment of sickle cell, leukemia, non-Hodgkin's lymphoma and some other cancers, life threatening anemias and auto-immune diseases. Current challenges with the use of stem cells in clinical practice include the provisions to direct the differentiation of embryonic stem cells into specialized cell populations, and also devise ways to guard their development or proliferation once placed in vivo. Only further research and its clinical application will solve the many unanswered queries.

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The first prenatal detection of an ovarian cyst was by Valenti in 1975. Since then antenatal and neonatal ovarian cysts are encountered more frequently due to the improvement of imaging techniques as well as routine antenatal ultrasound scanning. We discuss here the laparoscopic management of three cases of neonatal ovarian cysts. This approach is well tolerated by neonates, and it may overcome the controversy between the 'wait and see' policy and early surgical intervention, as laparoscopy has both diagnostic and therapeutic value with minimal morbidity, and ovarian salvage whenever possible.

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A frequently unrecognized and potentially devastating form of penile strangulation is that caused by human hair. Hair tourniquet syndrome frequently occurs during the time period when post partum mothers are experiencing increased hair loss. The majority of cases are thought to be accidental, however possibility of child abuse should be considered in every case.

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Retinoblastoma is the most common intraocular tumor in childhood. In majority of early stage retinoblastoma, the eyeballs as well as vision can be preserved with chemotherapy and local intraocular therapy with laser or photocoagulation. However, more than half the patients in India and other developing nations present in advanced stage of the disease. This article reviews the genetics, clinical approach, and treatment options for retinoblastoma focussing on advances in chemotherapy for intraocular retinoblastoma (chemoreduction), as well as improvement in survival in advanced retinoblastoma with surgery, chemotherapy, radiotherapy, and bone marrow transplantation.

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Postpneumonic empyema still remains quite common in developing countries, especially during the hot and humid months. While most cases would respond to antibiotic therapy, needle aspiration and intercostal drainage, few cases require further surgical management. The most common nontubercular etiological agent is Staphylococcus. Tubercular etiology is not uncommon in India, especially due to delayed presentation, multiresistant strains, mismanaged cases, and noncompliance with antitubercular treatment amidst malnutrition and anemia. Clinical symptoms, a skiagram chest followed by thoracentesis are enough for diagnosis. Pleural fluid is usually diagnostic and helps in choosing the appropriate antibiotics. Further investigations and management depends on the stage of the disease. Thoracentesis alone may be sufficient for the exudative phase. In fibrinopurulent stage, a properly sized and well-placed tube thoracostomy with underwater seal is curative in most cases. Interventional radiologists have placed small-bore catheters, specifically directed to the loculated collection and have used fibrinolytics like urokinase, streptokinase, and tissue plasminogen activator (TPA) to break loculations, ameliorate fibrous peel formation, and fibrin deposition. Thoracoscopic debridement and thoracoscopic decortication is an alternative with distinct advantages over thoracotmy and are indicated if there was no response with intercostal drainage procedure. In the organizing stage, a thoracotomy (for decortication) would be required if there is a loculated empyema, underlying lung disease or persistently symptomatic effusions. Timely institution of proper management prevents the need for any surgical intervention and avoids long-term morbid complications.

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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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Three infants who presented with clinical features of neonatal cholestasis and clinically suspected to have biliary atresia were found on imaging studies and surgery to have extrahepatic bile duct atresia in association with choledochal cyst. All patients were treated by bilio-enteric bypass procedure. Post operatively jaundice cleared in only two patients. The prognosis of this association depends on the pre-existent liver damage due to biliary atresia.

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Surgery for the correction of anorectal malformations (ARM) is performed by almost all pediatric surgeons. A number of operative procedures are practiced. The posterior sagittal anorectoplasty procedure has found wide acceptability and although it provides the most accurate anatomical reconstruction of the neoanorectum and the sphincters, the results are not in keeping with this technical advantage. In addition, there is no uniformity in describing the results of surgery and hence comparative evaluation of different series is difficult. This review describes the various methods that are available for the assessment of postoperative results following surgery for ARM, with a special emphasis on clinical methods keeping in mind the limitations in various parts of the country. Clinical examination and clinical scoring systems hold an important place in the post-operative evaluation of these patients. Imaging modalities are useful in the diagnosis of misplaced bowel and damage to the muscle complex and are necessary before re-do surgery is planned. Objectivity in the evaluation is provided by anorectal manometry combined with electromyography and these corelate well with clinical scoring systems. A consensus is required for uniformity in the methods of assessment.

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Recurrent parotitis is an uncommon condition in children. Its etiological factors have not been proved till date although causes due to genetic inheritance, local autoimmune manifestation, allergy, viral infection and immunodeficiency have been suggested. The exact management of this disorder is not yet standardized, but a conservative approach is preferred and all affected children should be screened for Sjogren's syndrome and immune deficiency including human immunodeficiency virus. We report a 12 years female child who presented with 12 episodes of non-painful recurrent swellings of the bilateral parotid gland in the past 3 years.

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We report a case of duplex kidney with Type-2 ureteral triplication, with one megaureter having stenosis at its lower end and dysplasia of the upper moiety of the kidney. The child presented with lump abdomen and recurrent attacks of urinary tract infection. The child underwent excision of the dysplastic part of the kidney and megaureter with an ureteroureterostomy connecting the normal caliber lower ureter (U3) draining in trigone with the mildly dilated middle ureter (U2) draining into the megaureter (U1) of the upper moiety. The patient is well 2 months postoperatively with no urinary infection and stable renal function.

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Background: The objective of the paper is to review the incidence and types of associated urogenital anomalies (U.G.A.) we encountered in patients with anorectal malformations (A.R.M.) and compare the results with previously published world literature. Materials and Methods: Retrospective review was done of 220 cases of A.R.M., treated from May 2002 to April 2003. All patients routinely underwent ultrasound (U.S.) study of the K.U.B. region and pelvis and lumbosacral radiography. Voiding cystourethrography (V.C.U.G.), nuclear renography and other investigations like buccal smear and karyotyping were done in selected cases only. Results: Genital anomalies were found in 30 cases (13.63%) and urologic anomalies in 25 cases (11.36%), a direct correlation being found between the level of A.R.M. and the incidence of urogenital anomalies (U.G.A.). Conclusion: The high incidence of associated anomalies (24.54%) makes careful clinical examination and evaluation of the urinary tract during the neonatal or early infantile period mandatory in all cases of A.R.M., particularly to avoid deterioration of renal function in future.

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The purpose of this study was to enquire into the clinical features and methods for the diagnosis and management of single-system ectopic ureters associated with renal dysplasia. Materials and Methods: A total of 13 female patients were studied. Main stay of diagnosis was ultrasonography of KUB region and intravenous urography and renal scan was used to confirm the diagnosis. Histopathological evaluation was done in all cases for documentation of renal dysplasia. Result: In eight cases ectopic ureter with dysplastic kidney was seen on left side and in five it was on right side. All the patients were treated with nephroureterectomy of the affected side because of poor functioning of ipsilateral dysplastic kidney. Conclusion: Continuous urinary incontinence in females with a normal voiding pattern should prompt an evaluation for ureteric ectopia and when initial evaluation yields diagnosis of solitary kidney the clinician should be aware of the possibility of a hypoplastic and/or dysplastic on one side and normally functioning kidney on opposite side. Nephroureterectomy is the treatment of choice for unilateral single system ectopic ureter with renal dysplasia of affected side.

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Positron emission tomography (PET) is a functional diagnostic modality, which is routinely used in oncology for diagnosis, staging, monitoring the response to therapy, and restaging of various tumors. PET used in conjunction with computerized tomography (CT) (PET-CT) is more useful as it provides anatomical details and the abnormal uptake on PET can be localized with highest accuracy. Much work has been done regarding PET/PET-CT in the adults but there is paucity in the same in pediatric group. A child must receive special attention when embarking on what is uncertain and often a frightening procedure. While performing PET in pediatric patients, emphasis is given to patient consent, intravenous access, bladder catheterization, and sedation. Based on existing literature and our own experience, we understand that PET-CT can play a very important role in initial staging, restaging, and evaluation of treatment response in lymphoma, neuroendocrine tumors and bone, and soft tissues sarcoma.

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Hepatic tuberculosis is usually associated with an active pulmonary or miliary tuberculosis, but rarely localizes as a liver tumor mass. The clinical presentation of isolated liver tuberculosis is so rare and atypical that it challenges the clinical acumen of the treating physician. Diagnostic modalities like ultrasound and computed tomography can miss the diagnosis. Ultimately, the diagnosis is confirmed by demonstrating an acid fast Mycobacterium in aspirated pus or necrotic material.

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