Ovarian Sarcoma a Diagnostic Dilemma- A Case Report.

Primary sarcoma of the ovary is extremely rare. There are inadequate data in the literature regarding ovarian sarcoma in the pediatric age group. We report a case of an 8-year-old girl presenting with large abdominal mass and cachexia. Raised alpha-fetoprotein levels suggested germ cell tumor. Tru-cut biopsy histopathological report suggested a spindle cell tumor. The IHC staining suggested non rhabdomyosarcoma. As tumour was large and ovarian pediatric non rhabdomyosarcoma was not reported in the literature, we started on rhabdomyosarcoma neoadjuant regimen. Good response was noted for neoadjuvant chemotherapy, which was followed by complete surgical excision of the tumor and radiotherapy. At present, the overall outcome of the disease is dismal. Increased available data and gaining more evidence may help in improvising the treatment option.

Vulvar Lichen Sclerosus Et Atrophicus: Refractory to Standard Therapy.

Vulvar lichen sclerosus et atrophicus is an inflammatory disease which can affect the pediatric age group, although rarely. Its prevalence is underestimated. It may have genital or extragenital manifestations. If left untreated, it may progress to complications which hamper the quality of life. The treatment of choice is topical clobetasol propionate. Here, we present a case of prepubertal vulvar LS, which was refractory to topical clobetasol but responded to topical tacrolimus application.

Effectiveness of structured physiotherapy in constipation in children with neurodevelopmental disorders-a randomized trial.

INTRODUCTION: Constipation is one of the major issues faced by children with neuro-developmental disorder (NDD). The aims of the study were to: 1) examine the effectiveness of a structured physiotherapy program on constipation in children with NDD; and 2) compare if conventional physiotherapy along with structured physiotherapy intervention has any combined effect on constipation in children with NDD. METHOD: Thirty-five children with neurodevelopmental disorder were assessed and randomly allotted into two groups. Twenty-two completed the intervention for 2 weeks and were statistically analyzed at baseline and post 4 weeks at a single tertiary center. The outcome measures used were Pediatric quality of life inventory (PedsQL), Peds QL Gastrointestinal symptoms scale, Bristol stool form scale, and defecation frequency. Group A received the conventional treatment, whereas group B received structured physiotherapy along with the conventional treatment. RESULTS: Group A had no significant outcomes, whereas in group B there were statistically significant differences for all outcome measures. Comparatively, a statistically significant change was noted for PedsQL GI symptoms scale (p = .045) and its constipation sub-scale (p = .002) in group B along with change in the Bristol stool form. CONCLUSION: Combined effect of structured along with conventional physiotherapy was better in terms of form of stool, constipation, and its associated quality of life factors as compared to conventional physiotherapy alone.

Solitary Peutz-Jeghers Polyp of Jejunum: A Rare Cause of Childhood Intussusception.

An extremely rare case of solitary jejunal Peutz-Jeghers polyp causing intussusception in an 8-year-old boy is reported. The polyp was excised by laparoscopic-assisted surgery. This appears to be only the fourth and the youngest patient with such a polyp reported in the indexed English language literature.

Is urodynamic evaluation a must in all operated cases of open neural tube defects.

BACKGROUND: Spina bifida is a common cause of pediatric neurogenic bladder. It causes renal failure in almost 100% of patients if the associated detrusor sphincter dyssynergia (DSD) is inadequately managed. Detrusor instability and high detrusor pressures (Figure) have been implicated as the major factors predictive of renal damage in these patients. Urodynamic studies provide early identification of "at risk" kidneys so that appropriate intervention can be made. However, the role in post-operative patients of spina bifida who have no clinical manifestations remains unclear. OBJECTIVE: To study the bladder dynamics in asymptomatic post-operative patients of spina bifida and to determine whether routine urodynamic study is justifiable. MATERIAL AND METHODS: Urodynamics was performed on 15 operated patients of spina bifida who did not have any neurological deficit and were asymptomatic. RESULTS: The mean age of the patients was 4.97 years. None of the patients had any urological complaints with their ultrasonography being normal. None had scars on nuclear scan. Of the 15 patients, 12 (80%) had abnormal findings on urodynamic assessment. Three patients (20%) had detrusor pressures greater than 40 cm of H2O. One patient had significant residual urine and detrusor instability. DISCUSSION: The use of urodynamic studies in asymptomatic patients of spina bifida remains controversial, with one school of thought advocating early invasive urodynamic testing. In contrast, some favor noninvasive sonological monitoring, reserving invasive tests only for patients with renal tract dilatation. In our subset of patients none had renal tract dilatation but three patients (20%) had "at risk" bladders. These patients would benefit from early intervention aimed at renal preservation. The study is limited by a small sample size because of the relative rarity of the patient profile included. A further multicenter study with a case-control design could conclusively indicate the role of urodynamic testing in these patients. CONCLUSION: Patients of spina bifida, even when asymptomatic, have a high incidence of unsafe bladders. Early identification and appropriate measures can protect kidneys from long-term damage, hence urodynamic profiling is mandatory for identification of potentially high-risk bladders.