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.

A Novel Technique of SMA Massage with Systemic Fibrinolytic Therapy in Ischemic Midgut Volvulus: As a Lifesaving Last Expedient.

Purpose: Midgut volvulus is a surgical emergency requiring immediate intervention. Intestinal ischemia of the midgut as a consequence of volvulus from malrotation is a fateful event with high mortality and significant morbidity even in survivors. Derotation followed by correction of malrotation is the procedure of choice though has significant morbidity if intestinal reperfusion was not successful. A combined treatment to restore intestinal perfusion based on the digital massage of the superior mesenteric artery after derotation and systemic infusion of fibrinolytic has been previously reported with success but underused. Here, we report three such cases of midgut malrotation with severe intestinal ischemia due to volvulus. Materials and Methods: A retrospective analysis of three confirmed cases of midgut malrotation with volvulus managed with emergency laparotomy, derotation, and Superior Mesenteric Artery (SMA) massage with systemic fibrinolytic therapy, followed by correction of malrotation was evaluated. Results: There was dramatic improvement in intestinal perfusion noted in all three patients inspite of delayed presentation. 2 out of 3 patients on follow up are doing well with adequate weight gain while 1 patient succumbed due to sepsis. Conclusion: Critical intestinal ischemia due to mesenteric thrombosis can persist after derotation of midgut volvulus and can lead to devastating consequences. The use of digital massage of SMA to disrupt the thrombus along with fibrinolytic therapy though reported is underutilized. Hence, awareness of this management and usage needs to be re-emphasized.

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.

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.

A Clinical Study on Congenital Diaphragmatic Hernia in Neonates: Our Institutional Experience.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a complex developmental defect having a multifactorial etiology; i majority of cases (~80%), the cause is not known. Survival rates for patients with CDH have increased over the past decade with early prenatal detection and better postnatal management including surgery. Clinical profile and the outcome of 83 CDH neonates were studied and analyzed over a period of 12 years in our institute. AIMS AND OBJECTIVES: The clinical study was to analyze the clinical profile and outcome of CDH among the neonates in a tertiary care referral neonatal and pediatric center in Karnataka, India. MATERIALS AND METHODS: This was a retrospective and prospective observational study conducted from January 2005 to March 2017, over a period of 12 years in a tertiary care referral neonatal and pediatric center in southern India. Clinical characteristics and risk factors of 83 neonates admitted and diagnosed with CDH were compared between survivors and nonsurvivors both preoperatively and postoperatively. Neonates with clinical and intraoperative diagnosis of diaphragmatic eventration were not included in this study. Multivariate logistic regression analysis was performed to determine independent predictors for mortality. RESULTS: A total of 83 neonates admitted and diagnosed with CDH were included in this study; 73 of them underwent surgical repair. The total survival rate in neonates with CDH was 70/83 (84.33%) and the overall operative mortality was 3/73 (4.1%). There was a significant difference between CDH neonates who survived 70/83 (84.33%) and those who died 13/83 (15.67%), in the age on admission, 5 min Apgar score, onset of respiratory distress, preoperative ventilation, the presence of persistent pulmonary hypertension of the newborn (PPHN), high-frequency oscillatory ventilation (HFOV), and length of hospital stay with P < 0.05. Using multivariate logistic regression analysis, the following factors independently predicted mortality: onset of respiratory distress in hours (odds ratio: 0.5, 95% confidence interval: 0.37-0.82) and preoperative ventilation (odds ratio: 0.02; 95% confidence interval: 0.0028-0.1558). When we compared CDH neonates who survived after surgery (n = 70) with those who expired (n = 3) postoperatively, there was a significant difference in the gestational age in weeks, side of CDH, PPHN, HFOV and length of hospital stay with P < 0.05. CONCLUSION: CDHs are common on the left side with fairly good prognosis. Though, the right-sided CDH are rare; they do carry a good prognosis, as it was seen in our experience.

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.

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.

Gastric Teratoma: An Unusual Presentation and Location.

The gastric teratoma is a rare tumor that usually presents as an abdominal mass, with or without features of gastric outlet obstruction. We report two cases of gastric teratoma; one - mature in a male neonate and another - ruptured immature gastric teratoma in a female neonate.

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.

Hepatic subcapsular flow: An early marker in diagnosing biliary atresia.

We report an interesting sign in the sonological evaluation of neonatal cholestasis, which is hepatic subcapsular flow. Hepatic subcapsular flow is an early and useful marker in diagnosing biliary atresia.

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.

Female form of persistent mullerian duct syndrome: Rare entity.

Persistent Mullerian duct syndrome (PMDS) is a rare form of Disorder of sex development in which Mullerian duct derivatives (fallopian tubes, uterus and the proximal vagina) are present in an otherwise normally differentiated 46 XY male. In the majority of cases, PMDS is a surprise finding either during orchidopexy or during inguinal hernia repair. We report a case of 4 year child with female type (Type III) PMDS. We are discussing the presentation, management and review of the literature.

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.

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.

Female Urethral Duplication: Rare Anomaly with Unusual Presentation.

Urethral duplication (UD) in females is a rare congenital anomaly and requires a high degree of clinical suspicion for diagnosis. The preoperative evaluation requires thorough investigations to delineate anatomy which is imperative for surgical reconstruction to provide excellent functional and cosmetic outcome. We describe the successful management of a 6-year-old girl with UD (presented as ambiguous genitalia and urinary incontinence) along with a review of pertinent literature.

Laparoscopic pyloromyotomy: Lessons learnt in our first 101 cases.

AIM: To analyze our experience with laparoscopic pyloromyotomy for infantile hypertrophic pyloric stenosis for the lessons that we learnt and to study the effect of learning curve. MATERIALS AND METHODS: This is a retrospective analysis of case records of 101 infants who underwent laparoscopic pyloromyotomy over 6 years. The demographic characteristics, conversion rate, operative time, complications, time to first feed and post-operative hospital stay were noted. The above parameters were compared between our early cases (2007-2009) (n = 43) and the later cases (2010-2013) (n = 58). RESULTS: 89 male and 12 female babies ranging in age from 12 days to 4 months (mean: 43.4 days) were operated upon during this period. The babies ranged in weight from 1.8 to 4.7 kg (mean: 3.1 kg). Four cases were converted to open (3.9%): three due to mucosal perforations and one due to technical problem. The mean operative time was 45.7 minutes (49.7 minutes in the first 3 years and 43.0 minutes in the next 3 years). There were 10 complications-4 mucosal perforations, 5 inadequate pyloromyotomies and 1 omental prolapse through a port site. All the complications were effectively handled with minimum morbidity. In the first 3 years of our experience the conversion rate was 9.3%, mucosal perforations were 6.9% and re-do rate was 2.3% as compared to 0%, 1.7% and 6.9%, respectively, in the next 3 years. Mean time for starting feeds was 21.4 hours and mean post-operative hospital stay was 2.4 days. CONCLUSION: Laparoscopic pyloromyotomy is a safe procedure with minimal morbidity and reasonable operative times. Conversion rates and operative times decrease as experience increases. Our rate of inadequate pyloromyotomy was rather high which we hope to decrease with further experience.

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.

Urethral ratio on voiding cystourethrogram: a comparative method to assess success of posterior urethral valve ablation.

OBJECTIVE: To develop a simple, objective and reproducible quantitative measurement to assess success of posterior urethral valve ablation. METHOD: In 30 patients with posterior urethral valves the diagnosis was confirmed by voiding cystourethrogram (VCUG). Our protocol was to perform valve ablation, and repeat VCUG at 12 weeks postoperatively. Urethral ratio was calculated by dividing the posterior urethral diameter by the anterior urethral diameter. Thirty males undergoing VCUG for urinary tract infections were evaluated as normative controls. RESULTS: Median age of controls was 12 months (2 days-6 years) and of study group was 13 months (1 day-11 years). Mean urethral ratio in pre-fulguration group was 4.94 (+/-2.97) and in post-fulguration group was 2.134 (+/-1.19) (P<0.001). The mean urethral ratio in the control group of 1.73 (+/-0.577) was significantly different from the pre-fulguration group result (P<0.001), but not significantly different in comparison to the post-fulguration group (P=0.104). CONCLUSION: Calculation of urethral ratio on VCUG as a method of assessment of outcome of fulguration is objective, reproducible, and allows preoperative and postoperative VCUG from different facilities to be compared. A post-fulguration urethral ratio of 2.5-3 represents an acceptable result postoperatively.