Isolated Sacrococcygeal Plexiform Neurofibroma in a Child.

This case report describes a 4-year-old girl with an isolated neurofibroma in the sacrococcygeal region. Although initially resembling sacrococcygeal teratoma, histopathology revealed a benign nerve sheath tumor. Wide local excision was performed, and the final diagnosis was plexiform neurofibroma. Diagnostic challenges in rare childhood tumors require stepwise evaluation and multidisciplinary team discussions.

Abdominal Wall Extraskeletal Ewing's Sarcoma in an 8-Year-Old Child.

Nonrhabdomyosarcoma soft-tissue sarcomas (NRSTSs) are a heterogeneous group of neoplasms of presumed mesenchymal origin. The precise diagnosis of the type of NRSTS tumor is complex. Among them, Ewing's sarcoma is very rare. We report a case of abdominal wall Ewing's sarcoma in an 8-year-old child. There are very few cases on pediatric abdominal wall Ewing's sarcoma reported. An 8-year-old female child presented with a progressively increasing swelling in the left iliac fossa for 3 months. On evaluation it was found to have a 4 cm × 2.87 cm × 4.1 cm circumscribed, heterogeneous exophytic mass arising from oblique muscles in the left lower abdominal wall with no intraperitoneal extension. On trucut biopsy and karyotyping, it was diagnosed to be an extraskeletal Ewing's sarcoma (EES) of the abdominal wall. Due to the proximity to the pelvic bone and the possible large size, the child was given six cycles of VAEC-IE as neoadjuvant chemotherapy to downsize the tumor. Following chemotherapy, wide excision of the tumor was done requiring partial resection of the external oblique, internal oblique, transverse abdominis, and leaving the transversalis fascia intact. The defect was closed using a polypropylene mesh. Histopathology showed tumor-free margins, with minimal histological tumor response to chemotherapy. Hence, the child underwent radiotherapy (45 Gy in 25 fractions). Follow-up fluorodeoxyglucose-positron emission tomography-computed tomography showed no recurrent lesion. There was no recurrence on 18-month follow-up. Extraskeletal Ewing's sarcoma is a rare tumor arising from the abdominal wall. The evaluation and management are similar to EES. Treatment is multimodal; however, surgery is the mainstay. Wide excision with tumor negative margin has a good outcome.

Long-term outcomes in children with Hirschsprung's disease and transition zone bowel pull-through: impact of surgical techniques and role for conservative approach.

PURPOSE: Presence of transition zone (TZ) in the pulled colon can impact the outcome of surgery in children with Hirschsprung's disease. There is a wide variation in terminology used to define TZ and its management. We present our series of managing 11 such children with considerations for conservative management. METHODS: Eleven of 114 children operated for Hirschsprung's disease had features of TZ on the 4-quadrant doughnut assessment of proximal anastomosing margin. They were followed up for development of obstructive symptoms, failure of pull-through procedure or bowel-related complications. Intervention done were observation with laxatives, dilatation, Botox injection and redo pull-through. RESULTS: Of the 11 children, 6 underwent Duhamel's procedure and 5, transanal endorectal pull-through (TERP). Features identified on HPE were presence of hypertrophic nerve bundles involving 2 or 3 quadrants in the circumferential doughnut biopsy of proximal anastomosing margin. Observed symptoms included constipation, enterocolitis, increased bowel frequency and soiling. Intervention done were use of laxatives with bowel management program in six and Botox injections in four. Only one child with TZ in 3 quadrants required redo surgery. Mean follow-up was 5.2 years with resolution of symptoms in most. CONCLUSION: This study highlights the role of conservative management with good outcomes in children with TZ bowel pull-through having hypertrophic nerve fibers and normal ganglion pattern. Children who underwent Duhamel's procedure had little impact with the presence of TZ at anastomotic margin and majority of those undergoing TERP benefitted from Botox injection. Conservative management can be attempted successfully to prevent redo surgical interventions as they can lead to poorer outcomes. Only those children not responding to conservative measures need to be planned for revision surgery.

Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon in a Neonate - Role of Dual Therapy: A Case Report and Review of Literature.

Kaposiform hemangioendothelioma (KHE) represents a rare, borderline vascular tumor with locally aggressive behavior. They are often associated with a potentially life-threatening coagulopathy known as Kasabach-Merritt phenomenon (KMP). Due to heterogeneous nature of the vascular lesion and lack of standardized treatment protocols, these patients pose a diagnostic dilemma and therapeutic challenge with morbidity and potential mortality. We report successful management of an infant with KHE and associated KMP. Difficulties encountered in diagnosis, initiation of therapy, and role of dual therapy with vincristine and steroids are discussed.

Intralesional Sclerotherapy with Bleomycin in Lymphatic Malformation of Tongue an Institutional Experience and Outcomes.

INTRODUCTION: The management of lymphatic malformations (LMs) continues to improve with advancement in molecular genetics, imaging, and treatment options. However, the management of tongue LMs remains a challenge due to the location, function involved, and long-term disabilities. We propose injection sclerotherapy with bleomycin in the management of spectrum of tongue LMs. METHODS: Children with LMs involving the tongue were prospectively treated with bleomycin sclerotherapy. Outcome measured was the efficacy of sclerotherapy, complications, and functional outcome. RESULTS: A total of 11 children underwent sclerotherapy with bleomycin for varying tongue lesions. Excellent outcome was seen in children with macroglossia. Eight children with isolated (focal) lesions had a resolution of symptoms with a clearance of lesions. Specific complications related to bleomycin toxicity were not encountered in our series during the follow-up of 4 years. CONCLUSION: In our series, children with macroglossia had an excellent outcome with normalization of tongue size and function. Children with focal tongue lesions also had good to excellent outcome. We recommend treatment of tongue LM with bleomycin sclerotherapy as the first line of management. Ease of treatment, early intervention, and excellent response makes it a favorable treatment option.

Penile Dislocation with Inversion: A Rare Complication of Blunt Pelvic Injury.

Penile injuries in children are usually uncommon and are predominantly associated with pelvic trauma or as postcircumcision injuries. The authors present a rare case of penile dislocation with penile inversion in a 5-year-old child occurring due to blunt pelvic injury. The child presented 3 months after pelvic injury with a suprapubic catheter for urinary diversion and absent penis with only penile skin visible. The presence of dislocated penile body was detected on magnetic resonance imaging, which was subsequently confirmed intraoperatively. During the surgery, the dislocated penis was identified and mobilized into its normal anatomical position within the remnant penile skin. Very few cases of penile dislocation have been reported in the literature. Pubic fracture with pulling of suspensory ligament resulting in dislocation of the penis would have been the probable mechanism of injury.

Role of synaptophysin in the intraoperative assessment of quadrantic innervation of the proximal doughnut in Hirschsprung disease.

BACKGROUND: Symptoms may persist in a retained aganglionic segment of the colon after corrective (pull-through) surgery in Hirschsprung disease (HD). Thus, it is important to assess the proximal doughnut for innervation abnormalities intraoperatively by frozen sections stained with conventional haematoxylin and eosin stain and supported by rapid acetylcholinesterase (AChE) histochemistry. When the doughnut is proximal to the sigmoid colon, AChE is not useful and requires ratification by yet another rapid technique and hence this study. METHODS: Two pathologists independently evaluated fresh doughnuts from the proximal bowel clinically assumed to be of normal innervation intraoperatively and chosen for anastomosis in patients with HD along with controls using AChE and synaptophysin (SY) immunohistochemistry. RESULTS: From 38 patients with HD, 28 doughnuts (63.7%) showed normal innervation with intense SY activity in the mucosa, the muscularis and the ganglion cells. The circumferential aganglionic doughnuts (abnormal innervation) (n= 6, 13.6%) showed neither SY-positive fibres in the mucosa nor in the muscularis. The abnormal transition zone doughnuts (n=10, 22.7%) showed involvement of three quadrants of the doughnut in one, two quadrants in three and one quadrant in six with decreased SY-positive fibres in the muscularis and scattered ganglion cells with a statistically significant measure of agreement of (κ=0.973) between the two. CONCLUSION: The pattern, intensity and distribution of SY-positive fibres in the muscularis propria of the doughnut of the proximal bowel chosen intraoperatively for anastomosis in HD can identify sectors with abnormal innervation allowing the surgeon to seek normal innervation status more proximally to avoid complications.

Ruptured rectal duplication with urogenital abnormality: Unusual presentation.

Rectal duplication (RD) accounts for 5% of alimentary tract duplication. A varied presentation and associated anomalies have been described in the literature. Antenatal rupture of the RD is very rare. We present an unusual case of a ruptured RD associated with urogenital abnormalities in newborn male. We are discussing diagnosis, embryology, management and literature review of ruptured RD.

Skip segment Hirschsprung's disease: a case report and novel management technique.

Skip segment Hirschsprung's disease is a condition where an area of normally ganglionated intestine is interspersed proximally and distally by aganglionic segments. The occurrence of skip segment has no clear embryological explanation, contrary to the present concept of failure of cranio-caudal migration of neural crest-derived cells in Hirschsprung's disease. Only 25 case reports have been published in literature so far and this is possibly the first to be reported from Asia. We report one such case with novel surgical management.

A Cross-Sectional Study of Hypertension among 20-40 Years Old Residing in an Urban Area of Bagalkot City, North Karnataka.

Introduction: Hypertension is the commonest cardiovascular disorder and one of the major risk factors for cardiovascular mortality which accounts for 20-50% of all deaths. Although most studies describe hypertension in older adults and the elderly, there is a paucity of data on young adults as they are deemed to be at a lower risk of developing the disease. Objectives: To study the prevalence of hypertension and study the risk factors and strength of association between factors and hypertension among 20-40 years old. Methodology: A cross-sectional study was conducted among 20-40 years old residing in the urban field practice area to find the prevalence of hypertension and its association with socio-demographic factors. After obtaining informed consent, data were collected with the help of an interview method by systematic random sampling using predesigned and pretested semi-structured questionnaires. Three readings of blood pressure were recorded using a sphygmomanometer, and the average reading was considered. Data analysis was done using Microsoft Excel, Open Epi software, and SPSS software. Results: Out of 420 subjects, the prevalence of hypertension was found to be 17.9% and pre-hypertension was 52%. Factors significantly associated with hypertension were age, marital status, type of family, family history of hypertension, tobacco chewing, stress, and body mass index (BMI). Conclusion: The present study reveals a high prevalence of hypertension (17.9%) in the 20-40 years old age group, which can lead to increased cardiovascular disease burden in the population.

Estimation of plasma and RBC acetylcholinesterase in children: An evaluation tool for Hirschsprung's disease?

BACKGROUND: Increased acetylcholinesterase (AChE) activity on frozen sections of rectal mucosal biopsies accurately diagnoses Hirschsprung disease (HD). But the quest for a biomarker in blood as a screening test prompts one to look for AChE in blood and study its role in HD diagnosis. AIM: To develop a low-cost reliable method to estimate the AChE activity in plasma and red blood cells (RBCs) in normal children (control) and study its role in HD (test). MATERIALS AND METHODS: Optimized method derived after modifying and standardizing known AChE assay protocols for blood were employed on 30 controls to define the AChE cut-off range, on 40 suspected HD cases to categorize them as HD/non-HD based on cut-off values and later compared with gold standard tissue AChE histochemistry of rectal mucosal biopsies. RESULTS: An optimal in-house modified methods of Ellman's was found best suited to analyze plasma AChE activity, method by Wilson and Henderson was optimal for extraction and AChE estimation in RBCs. AChE levels (controls) obtained were 1.03 ± 0.31 U/mL and 5.17 ± 1.52 U/mL in plasma and RBCs, respectively while the plasma AChE was 1.35 ± 0.84 U/mL (HD) and 1.62 ± 0.85 U/mL (non-HD) while RBC AChE was 4.29 ± 3.2 U/mL (HD) and 6.48 ± 4.31 U/mL (non-HD). Sensitivity was 66.67% and 55.56%, specificity was 22.73% and 45.45%, and an accuracy rate of 42.5% and 50% for plasma and RBC, respectively. CONCLUSIONS: Mutually exclusive AChE activity range identified for test blood samples overlapped with the normal and hence, not considered a diagnostic tool for HD.

Pediatric empyema thoracis management: should the consensus be different for the developing countries?

BACKGROUND: Despite the available consensus, intrapleural fibrinolytic therapy (IFT) in pediatric empyema is grossly underutilized in the Indian subcontinent where the disease burden is huge. Possible reasons may be epidemiological differences and physician bias. There is a paucity of literature from developing countries on the use of IFT in pediatric empyema thoracis. Hence, this study was undertaken to determine if fibrinolytic therapy is equivalent to video-assisted thoracoscopic surgery (VATS) in treating stage II empyema in children even in developing countries. METHODS: Consecutive cases of stage II empyema were randomized to receive either IFT or VATS. The outcomes measured were the duration of hospital stay, efficacy of therapy, complications, and cost differences. RESULTS: 41 children were randomized to either VATS (n = 20) or IFT (n = 21) group. Overall successful clearance of empyema was achieved in 18 out of 20 (90%) children undergoing VATS and 20 out of 21(95.2%) children in fibrinolytic arm. The median length of the hospital stay was 7 and 8 days for VATS and IFT groups respectively (p = .24). Need for CT scan and blood transfusion was significantly higher in the VATS group than IFT group (p = .02 and .000). CONCLUSIONS: Fibrinolytic therapy is noninferior to VATS in the treatment of stage II empyema in children in the Indian subcontinent. A multicenter trial with larger sample size and uniform, detailed protocols on indications for CT scan, blood transfusions, nutrition status and costs involved will be needed to eliminate institutional bias and to increase the strength of the study. STUDY TYPE: Randomized controlled study, treatment study and cost effectiveness study.

Thoracoscopy in stage 3 empyema thoracis in children - A safe and feasible alternative to thoracotomy.

BACKGROUND: Even though the role of thoracoscopy is well defined in Stage 2 empyema thoracis and is very popular, its role in the management of advanced empyema is still unclear. The technical difficulties and the potential complications are the principal reasons for the hesitancy in attempting video assisted thoracoscopic surgery (VATS) in advanced stages. METHODS: We prospectively studied the safety, feasibility and effectiveness of VATS for decortication in Stage 3 empyema. RESULTS: In the 61 cases that we attempted VATS over the last 7 years, we could complete the procedure in 45 patients (73.77%). Four children among them required re-do procedure later for persistent problems. Conversion to thoracotomy was needed in 16 patients (26.23%). The post-operative hospital stay of patients who underwent primary VATS decortication was significantly less when compared to patients requiring conversion (p < 0.0001). CONCLUSION: Thoracoscopy is a safe, feasible and effective option even in advanced empyema thoracis and should be offered in centers with adequate expertise and set up. LEVEL OF EVIDENCE: Level II.

Technical modifications of laparoscopically assisted anorectal pull-through for anorectal malformations.

PURPOSE: Our technique of fistula ligation and centering of bowel during the laparoscopically-assisted anorectal pull-through (LAARP) for the high-imperforate anus is described. METHODS: The distal rectum is dissected laparoscopically. About 1 in proximal to the termination of the rectum, we commence a subseromuscular dissection to create a mucosal tube of the distal rectum up to the urethra. This mucosal tube is then ligated and sharply divided. Under laparoscopic guidance, the needle is inserted between the two bellies of Levator Ani muscle, just posterior to the urethra. Simultaneous external stimulation confirms the optimal position. A guide wire is then passed through the needle. The tract is serially dilated and the anoplasty completed. RESULTS: This technique has overcome the problem of residual urethral diverticulum in our cases.

Double-barrel common bile duct: a rare cause of extrahepatic biliary obstruction.

Double-barrel common bile duct is rare. We report a 50-year-old woman with defective canalization of the common bile duct, presenting with extrahepatic biliary obstruction due to stones in one compartment. CT scan highlighted this anomaly. After failed attempts at stone extraction at ERCP, she was successfully operated on.

Management of Bilateral Wilms' Tumor: Our Experience.

Management of bilateral Wilms' tumor is particularly challenging, considering the chances of recurrence and long-term renal function for affected patients. Aggressive surgical resection to prevent recurrence must be balanced with the desire to preserve renal function. We evaluated our experience in the management of bilateral Wilms' tumor stressing the challenges encountered in decision making and the role of nephron sparing surgery. We had four children presenting with bilateral Wilms' tumor. All of them were appropriately staged and given standard chemotherapy as per NWTS-5 guidelines. Tumors were considered to have a 'good' response to chemotherapy if sufficient tumor shrinkage was observed so that renal hilum was seen free of tumor and vice versa. Nephron-sparing surgery was considered in all and was performed when feasible, followed by completion adjuvant chemotherapy. All patients were followed up with serial ultrasound scans (3-6 monthly) and CECT abdomen (yearly once). Blood urea and serum creatinine, hypertension, and proteinuria were assessed during follow-up visits. All four children received neo-adjuvant chemotherapy as per NWTS-5 guidelines. The first child had poor response to chemotherapy and was considered for left radical and right partial nephrectomy. However, patient attenders refused any surgical intervention and the child was taken home. The second child had a 'good' response on left side and was planned for left partial and right radical nephroureterectomy based on pre-operative imaging analysis. However, intra-operatively, bilateral partial nephrectomy with good margins was feasible. The third child though, showed 'poor' response to pre-op chemotherapy and warranted bilateral nephroureterectomy, right partial and left radical nephroureterectomy was feasible. However, in the fourth child, we were not able to perform nephron sparing surgery and left nephroureterectomy with right tumor biopsy was done. Following this, child was started on 2nd-line chemotherapy, now awaiting right partial nephrectomy. The second child is on follow up for 1.5 years, doing well. However, the third child expired 1.5 years following surgery due to recurrence (lung metastasis). Management of bilateral Wilm's tumor is challenging and nephron-sparing surgery should be considered in all patients having bilateral Wilm's tumor with favorable histology, even if pre-operative imaging studies suggest that the lesions are unresectable.

Totally Implantable Venous Access Devices in Children Requiring Long-Term Chemotherapy: Analysis of Outcome in 122 Children from a Single Institution.

Children with malignancy require venous access that is reliable, safe and compliant on a long-term basis. There is little data available on utilization of totally implantable venous access devices (TIVAD) for long term chemotherapy in children in an Indian setting [1]. We report our long-term follow-up results of utilization of totally implantable venous access devices for long-term chemotherapy in children. This was a retrospective analysis of 122 children requiring long-term chemotherapy done between January 2008 and December 2013. Data collected included primary disease process, type of port, site of insertion, intraoperative events, early and late postoperative complications, and issues with utilization, maintenance and removal. 127 ports were placed in 122 children. The follow up ranged from 16 to 50 months. Internal jugular vein was accessed in 96.8 % of cases (123/127). Majority of children (61 %) had hematological malignancy. Early complications occurred in 5 children. Late complications occurred in 18 children which included port pocket infection in 3, port site skin issues in 5, catheter related issues in 3, venous thrombosis in 2 and catheter related bacteremia in 5 children respectively. Only 10 children have been lost to follow-up either due to death or discontinuation of treatment and rest are on follow up. Totally implantable venous access devices usage is safe and reliable for access needs in children for long-term chemotherapy. Their low complication and low cost maintenance should increase their utilization in children requiring long-term chemotherapy. Chemoport placement in children with hematological malignancy can be carried out safely without much impact on complication rates. Though management and compliance of children with malignancy has improved; critical analysis and standardization of port system care through prospective trials are necessary to reduce the morbidity and for cost analysis in these children.

Continent catheterizable conduit for urinary diversion in children: Applicability and acceptability.

BACKGROUND: Continent catheterizable conduit (CCC) has made clean intermittent catheterization (CIC) painless and easy. It is applicable in diverse clinical conditions. Nonetheless, convincing the parents for the need of conduit procedure is still difficult. MATERIALS AND METHODS: A prospective study, included children who underwent CCC procedure from March 2008 to February 2013. The data were assessed for; diagnosis, type of conduit, number of preoperative counselling sessions before acceptance, role of "self-help group" in decision making, parental concern and satisfaction for the procedure. RESULTS: Twenty-nine patients (males; 24, females; 5) underwent CCC procedure for various clinical conditions. The multiple preoperative counselling sessions and creation of "self-help groups" were helped them for decision making. The main concerns among parents were: (1) Impact of procedure on future fertility and sexual life. (2) Patency of native urethral channel. (3) Permanent urinary stoma over the abdomen. CONCLUSION: CCC procedures are applicable to a wide array of clinical situations with a good outcome. The acceptability of the CCC procedure improves with preoperative counselling of parent/child, initiation of preoperative per urethral CIC and creation of self-help groups.

Complications of chemoport in children with cancer: Experience of 54,100 catheter days from a tertiary cancer center of Southern India.

BACKGROUND: Chemoport is an essential part of the management of children with cancer and provides long-term venous access. There are few studies from resource poor countries reporting complications of chemoport. AIMS: This study was aimed at describing the complications of chemoport in patients with cancer. MATERIALS AND METHODS: This retrospective observational study analyzed 200 patients <15 years of age who underwent chemoport insertion. The medical records of these patients were reviewed for the patient characteristics, diagnosis, nature of port use, port-related complications and their management. RESULTS: A total of 209 ports were implanted in 200 patients and 24 ports were removed due to port-related complications. There were 122 boys and 78 girls whose ages ranged from 4 months to 13 years (median age 2.5 years). About72% of patients were <2 years old. The cumulative duration of catheterization was 54,100 days. Of 209 ports, there were 36 complications that led to the removal of 21 ports. Port-related infection was the most common infection observed in our study (0.66/1000 catheter days and 11.9%). Mechanical complications were seen in 9 patients. Venous thrombosis and skin necrosis occurred in one patient each. CONCLUSIONS: Use of chemoport is safe and is a boon for children with cancer in developing countries with incidence of complications similar to Western countries. Although use of chemoport is associated with complications, they are easily managed. With stringent catheter care by trained personnel, some complications can be prevented.