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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Acute appendicitis is a common occurrence in children. The current trend is to perform appendicectomy once acute appendicitis is diagnosed. The aim of this study was to evaluate the types of pathology seen in appendices removed for acute appendicitis and correlate them with clinical features. The clinical, operative and histological records of 302 children who had appendicectomy at the Jos University Teaching Hospital from 1995-2003 were retrospectively reviewed. There were 123 (40.7%) boys and 179 (59.3%) girls (m: f = 1: 1.5). Their ages ranged from 2-15 years (median: 12 years). The main symptoms and signs at presentation were right lower quadrant abdominal pain, anorexia, nausea, vomiting, right quadrant tenderness, guarding and rigidity. Clinical impressions included simple acute appendicitis in 260 and perforated appendicitis in 42 patients. Of the 302 children who had clinical diagnosis of acute appendicitis, only 144 (47.7%) were confirmed at histology. Lymphoid hyperplasia was the most common pathology seen in the appendix specimens. The clinical impressions of acute appendicitis made by the surgeon agreed with pathologists' report in 63.9% and disagreed in 36.1% of cases. Negative appendicectomy rate was 52.3%. Although appendicectomy is the most commonly performed emergency abdominal surgery in children, the procedure is still associated with a high negative appendicectomy rate.

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Aim: We present here experience of a single surgeon with Mitchell's procedure for correction of epispadias. Materials and Methods: Nine boys (mean age 5½ years, range 9 months to 16 years) underwent Mitchell's repair in Department of Pediatric Surgery over a period of 5½ (September 1999 to March 2005) for correction of epispadias. Six of these patients had come for the second stage of exstrophy-epispadias repair after primary bladder closure; the other three had incontinent penopubic epispadias. Results: The penis was cosmetically acceptable as regards to size, glans shape and peno-pubic angle in all the patients. However, there was a high incidence of penopubic fistula (44%). These patients with penopubic fistula also required postoperative urethral dilatations, at times repeated. One of the common factors to these subset patients was their younger age when Mitchell's urethroplasty was performed. Limitations: The series is descriptive in nature, short in numbers and does not provide statistical comparison of Mitchell's procedure with the previously done procedures. Conclusions: Mitchell's complete penile disassembly technique for epispadias repair is more acceptable anatomical procedure that results in near-pendulous penis. However, when performed at young age, it is fraught with the complication of penopubic fistula similar to that as seen with Cantwell-Ransley's procedure. Mitchell's procedure creates a hypospadiac meatus initially and the meatal advancement is required as for any other distal penile/coronal hypospadias.

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Abdominal cerebrospinal fluid (CSF) pseudocyst is an uncommon complication following ventriculo-peritoneal (VP) shunt. The following report's our experience with three cases of CSF pseudocyst in children. VP shunt was done earlier for communicating hydrocephalus following tubercular meningitis (TBM) in all cases. Clinical presentation was with progressive abdominal distension and features of intestinal obstruction. Clinically we were able to diagnose all cases as CSF pseudocyst peritoneal cavity. Ultrasound examination confirmed the clinical findings in all. CT scan of abdomen and pelvis showed a large unilocular CSF pseudocyst with shunt catheter within it on one patient (case 3). Ultrasound guided aspiration of cyst was done in case 1 alone, but failed to resolve the symptoms. All patients needed formal exploration. Near total cyst excision, adhesiolysis and relocation of peritoneal end of VP shunt catheter in right supra hepatic space was done in all. Two patients who developed shunt tract infection needed shunt removal. The follow up period is 6-8 months.

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Background: Definitive treatment of isolated esophageal atresia (IEA) is still controversial. A study was conducted to review cases of IEA in our department with a view to evaluate the long term results of definitive surgery in these patients. Materials and Methods: Nine consecutive patients with IEA were included in the study. All babies initially underwent cervical esophagostomy and feeding gastrostomy. One baby also had anorectal atresia and needed a colostomy. In 6 babies, end esophagostomy was performed while in 3 babies, lateral esophagostomy was performed. Of the 6 babies with end esophagostomy, two underwent jejunal Interpositioning, two had serial extra-thoracic lengthening and two reverse gastric tube interpositiong. Three babies with lateral esophagostomy were planned for delayed primary repair. Results: Both babies with jejunal interpositiong initially did well; serious gastro-esophageal reflux occurred in one baby who needed antireflux surgery. At 6 years follow-up both children had redundant Jejunum. Recurrent Respiratory tract infection remained a problem in one child while other is doing well. Of the two babies with extrathoracic lengthening it was possible to perform end to end anastomosis in one baby but in the other extrathoracic lengthening did not work and gastric transpositioning was performed. Of the two babies with reverse gastric tube interpositioning one developed esophageal stenosis at the site of anastomosis and is on serial dilatations. Other died due to anastomotic leak and mediastinitis. In all the three babies with lateral esophagostomy spontaneous lengthening of esophagus was noted. End to end anastomosis was possible in two of these babies who are doing well. One is waiting definitive surgery. Number of complications, hospital stay and cost of surgery was least in patients with lateral esophagostomy. Conclusion: Various methods of definitive treatment have been proposed for isolated esophageal atresia but each having its own complications. Treatment therefore has to be individualized. Lateral esophagostomy with spontaneous esophageal growth may be a good option in developing countries with limited facilities.

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We report an 18-month-old boy who presented with acute organoaxial volvulus of stomach secondary to eventration of diaphragm. Clinically, the child exhibited classical triad of epigastric distension, unproductive retching and inability to pass a nasogastric tube. Successful operative management is presented and relevant literature is discussed.

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The aim of the study is to investigate the significance of spot urine 5-hydroxyindoleacetic acid (5-HIAA) levels in patients admitted with the suspicion of acute appendicitis. Seventy-one patients with the mean age of 9.4±2.9 years, who were admitted to our pediatric surgery clinic between August 2002 and March 2004 with the complaints of abdominal pain were evaluated prospectively. Additionally spot urine samples were collected from 34 healthy children for control. 5-HIAA was detected from the urine samples in all children with high-performance liquid chromatography (HPLC) method. The results were analyzed with one way analysis of variance (ANOVA), post hoc Tukey HSD test and receiver operating characteristic (ROC) curve. After the clinical follow-up appendicitis was detected in 40 patients and abdominal pain was found to be due to causes other than appendicitis in 31 patients. Results were found to be correlative with the final diagnosis in all patients. The mean 5-HIAA levels were 2.5±1.8 µmol/dl in healthy children, whereas 9±5 µmol/dl in nonappendicitis and 18.9±17.8 µmol/dl in appendicitis patients, respectively. The difference between the patients with acute appendicitis and the other two groups were statistically significant ( P = 0.001). For a value of 8.9 µmol/dl, this test was found to be 70% sensitive and 67% specific according to the ROC curve. 5-HIAA significantly rises in pediatric acute appendicitis. However, due to high values of SD and relatively low sensitivity and specificity, this test seems to have limited diagnostic power as a single parameter in childhood acute appendicitis.

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In cases of repair of urethrocutaneous fistulae developing after primary hypospadias surgery, tubularisation often becomes difficult after excision of scar tissue. It is also well-known that this type of fistulae has a high recurrence rate. In this study of 15 cases, the aim is to analyze the results of conventional urethrocutaneous fistula repair, augmented with the principle of Midline longitudinal incision of the dorsal wall of the urethra at the site of the fistula according to the concept of Tubularised Incised Plate Procedure with the hope of preventing recurrence. Here, the excision of scar tissue around the urethrocutaneous fistulae, tubularisation of urethra is done after incising the dorsal urethral plate. Then the mucosa is closed over a 6F/8F catheter and the resultant suture line is covered first with the dartos flap or adjacent tissue and then, the skin. The skin layer is minimally rotated so as to avoid suture-line overlap. Results of this method has been found to be quite satisfactory, as only in two[2] patients the fistula recurred. Thus it may be concluded from this study that the Tubularised Incised Plate Urethroplasty along with conventional urethral fistula repair is a good adjunctive procedure in prevention of their recurrence.

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The rupture of an omphalocele sac during birth is a well recognized entity. The associated lesions due to vascular compromise can result in necrosis of the bowel with perforation. Spontaneous bowel perforation in an omphalocele at birth is not reported in the literature. We describe a case with bowel perforation at the fundus of an omphalocele in a newborn.

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