Neuroblastic tumours in children: Outcome from a referral centre in India.

Background: There is a paucity of uniform literature on the outcome of children with neuroblastic tumours from developing countries. This study aims to present the outcome in children having neuroblastic tumours. Materials and Methods: We retrospectively reviewed patients with neuroblastic tumours from January 2014 to March 2020. Data analysed were pertaining to the age, sex, presentation, site, stage, diagnostic evaluation performed, management and follow-up results, etc., The final outcomes were assessed as complete response; partial response (PR); no response (NR) and progressive disease. International Neuroblastoma Risk Group staging was used and patients were categorised on the basis of age, site and stage of tumour. Overall survival (OS) was calculated from the date of diagnosis to the date of last follow-up and event for OS was death. Results: Forty-seven patients were included with median age of 60 months (ranges of 2-180; mean = 61.87 ± 47.56). About 72.3% (n = 34) patients were males. Out of total, 6.4% (n = 3), 53.2% (n = 25) and 38.3% (n = 18) were in stage L1, L2 and M, respectively, whereas 2.1% (n = 1) patients were in stage MS. Surgery was performed in 25 (53.19%) patients, but gross total excision was achieved in 48.90% (n = 23) patients. Onlu 10.60% (n = 5) patients were referred, whereas 14.89% (n = 7) patients reported recurrences. Mean and median follow-up time of the patients was 24.64 ± 16.04 and 18 months (range: 3-60 months). Out of total, 53.2% (n = 25) and 29.8% (n = 14) patients had complete and PRs, respectively, whereas 17% (n = 8) patients had NR. Out of the total 47 patients, 8 (17%) achieved events (deaths), whereas the rest, 39 (83%) patients, were censored. Mean event-free OS time was 50.04 months. Conclusion: There was a significant difference in patient deaths in recurrence and non-recurred patients (4/7, 57.1% vs. 4/40/, 10%, P = 0.011). Survival time was significantly higher in patients with stages L1-L2 as compared to Stage 4. Stage and age were found predictors of survival.

Is application of salt for 3 days locally is sufficient to treat umbilical granuloma?

BACKGROUND: The falling of Umbilical stump occurs by 7-15 days of age. The healing of umbilical stump may be complicated by Umbilical Granuloma. It is often treated by chemical cauterisation which require repeated applications and may lead to local or systemic complications. Common salt by way of its dessicative property may help in treatment of Umbilical Granuloma. OBJECTIVE: The objective of the study is to assess the role of common salt application in umbilical granuloma. MATERIALS AND METHODS: This is retrospective study over 3 years from a pediatric surgery unit in Northern India. The study subjects were infants less than 10 weeks of age who presented with umbilical granuloma. The method of salt application was 1 pinch of common salt for 1 hour twice a day for 3 consecutive days. The babies were assessed at day 5th for resolution. The success was defined as thrice resolution after 3 cycles. The baseline demographic details were taken and the association of success of treatment was analyzed. RESULTS: A total of 36 infants were given treatment in form of common salt application for treatment of umbilical granuloma. The success of around 96% and the cases which presented early responded well. Most of the cases resolved after 3 cycles of treatment. CONCLUSION: The common salt application is effective in treatment of granuloma without any side effects.

Constipation after surgery for anorectal malformations: Unrecognised problem until it is a problem.

BACKGROUND: Constipation is a common problem after surgery for anorectal malformations (ARMs), especially in patients having preserved rectosigmoid after pull-down surgery. Here, we present our experience with patients having constipation after surgery for ARMs and briefly discuss its management. MATERIALS AND METHODS: We retrospectively reviewed electronic operative and follow-up data from January 2015 to December 2019 of all patients having constipation after surgery for ARMs. RESULTS: A total of 77 patients were included in the study. The median age was 60 months, and thirty patients were male. Primary diagnoses were rectourethral fistula (26; 33.77%), vestibular fistula (25; 32.47%), perineal fistula/ectopic anus (18; 23.38%), rectovaginal fistula (4; 5.19%) and imperforate anus without fistula (4; 5.19%). Anal stenosis was found in 20 (25.97%) patients, posterior ledge with an adequate anal opening in 5 (6.49%), displaced anus in 4 (5.19%) but no obvious problem found in 48 (62.34%) patients. Non-operative management was successful in 75.44% (43 out of 57) of patients. Surgery was performed in 32 out of 77 patients (41.56%) with no mortality. Forty-three (72.88%) patients are continent, stayed clean, but 16 (27.19%) patients still need some sort of laxative/bowel wash/enema intermittently (Grade I/II constipation). Incidence of constipation was higher in patients operated through anterior sagittal route (27.58%) than posterior sagittal route (23.94%), but it was not statistically significant (P = 0.479). Follow-up ranged from 3 months to 5 years. CONCLUSION: Constipation is a common problem after ARMs surgery, especially in patients having preserved rectosigmoid after pull-down surgery. It is advisable to create relatively larger neoanus after pull through with early practice of neoanus dilatation, especially in redo cases. Repeated counselling, laxatives and dietary manipulations should begin at the first sign of constipation.

Congenital urethrocutaneous fistula: Case report with review of literature.

We are presenting two cases of congenital urethrocutaneous fistula on ventral penile shaft. Congenital urethral fistula is an extremely rare, but easily manageable anomaly that may be confused with hypospadias. Awareness of the entity will avoid complications. This condition may be associated with other anomalies like congenital hernias and anorectal malformations. Treatment of this entity is individualized according to site of fistula, associated anomalies and condition of the distal urethra. All the principles of hypospadias surgery should be strictly followed.

Tracheoesophageal fistula with esophageal atresia with absent external ear: a unusual association.

Esophageal atresia with tracheoesophageal fistula (TEF with EA) is a common disease which is associated with many other congenital abnormalities. The most common association is with VACTERL syndrome.. Various types of ear malformations have been reported in CHARGE syndrome associated with EA and TEF. However, absence of external ear has not been described which is being reported in this article. We could not found this association even on extensive search of literature.

Duplication cyst of pyloroduodenal canal: a rare cause of neonatal gastric outlet obstruction: a case report.

BACKGROUND: A 21 day old male child presented with non bilious vomiting and abdominal mass. CASE PRESENTATION: This case is reported because pyloroduodenal duplication cysts are an extremely rare congenital anomaly, whose clinical presentation often mimics those of hypertrophic pyloric stenosis. Ultrasound examination showed cystic mass at pyloric region and barium study was suggestive of extrinsic mass compressing the pyloric region. A laparotomy, a tense cystic mass was present at the pyloroduodenal junction (PDC) which was resected and end to end anastomosis was done. Patients followed an uneventful recovery and doing well. CONCLUSION: The clinical and radiological analysis can reveal configurational changes consistent with a large extrinsic mass rather than muscular hypertrophy and can lead to accurate preoperative diagnosis.

Histology of the terminal end of the distal rectal pouch and fistula region in anorectal malformations.

OBJECTIVE: Until recently, surgeons have been posed with a dilemma---whether or not they should preserve the terminal end of the distal rectal pouch and the fistula region in anorectal malformations (ARMs). A detailed histological study of this region was conducted to establish a consensus for preserving or excising this region for reconstruction of ARMs. METHODS: Histopathological examination using haematoxylin and eosin-stained sections of the terminal portion of the distal rectal pouch and proximal portion of the rectourogenital or rectoperineal connection was performed in 60 cases of high, intermediate and low ARMs. RESULTS: Distorted internal sphincter was present in 93.3% of high, 90% of intermediate and 100% of low ARMs. The proximal fistula region was lined by transitional epithelium in 50% of cases, and anal glands were present in 83.3% and anal crypts in 68.3% of cases. The rectal pouch in the region of the internal sphincter and fistula was aganglionic in all cases. CONCLUSION: This study shows that the terminal end of the distal rectal pouch and proximal fistula region possess distorted anal features with aganglionosis, and contradicts the recommendation that this region should be reconstructed in patients with malformations.

Anterior intratumoural chemotherapy: a newer modality of treatment in advanced solid tumours in children.

OBJECTIVE: Advanced and inoperable solid tumours in children have high mortality despite aggressive multimodal treatment. Intravenous chemotherapy is abandoned at times because of systemic toxicity. This study investigated intratumoural chemotherapy and compared it with intravenous chemotherapy. METHODS: Forty children with advanced inoperable solid tumours (Wilms' tumour and neuroblastoma) were randomly allocated into two groups of 20. Group A was given intratumoural chemotherapy and group B was given intravenous chemotherapy. Both groups were compared for reduction in tumour size and volume, tumour resectability, histopathological changes and drug side effects. RESULTS: Intratumoural chemotherapy was superior to intravenous chemotherapy in terms of reducing tumour size and volume (63% in group A vs. 22% in group B). The resectability was 70% in the intratumoural group compared with 40% in the intravenous group. The overall good histopathological response was 71% in group A as opposed to 0% in group B. Moreover, the incidence and severity of drug side effects and morbidity were less with intratumoural chemotherapy. Mortality was also low in group A (5%) compared to group B (20%). CONCLUSION: Intratumoural chemotherapy can be offered as an effective and safe alternative treatment modality for advanced and inoperable Wilms' tumour and neuroblastoma.

Neonatal surgery: a ten year audit from a university hospital.

Neonatal surgery is the flagship and most challenging component of pediatric surgery, which is the youngest subspeciality of surgery. Neonatal surgery carried a survival rate of only 30% three decades ago. In the last decade there has been a significant change in the scenario. Earlier recognition and referral of these anomalies, availability of neonatal intensive care, better preoperative planning, decision, and techniques have lead to the change in the management. This is an audit into the outcome of neonatal surgery from one of the largest units in India over a ten year period. This audit reveals an across the board survival of 65-70% newborns after surgery on nearly two thousand case over a ten year period. It has an important message that while pediatric surgery units expand, risk stratification of surgical newborns and their treatment in suitable units is mandatory to maintain and improve these figures to match international standards over the next decade.

Use of fibrin glue in preventing urethrocutaneous fistula after hypospadias repair.

UNLABELLED: Urethrocutaneous fistula is one of the most common complications after hypospadias surgery. The incidence of fistula development has varied from 4% to 20% in larger series. We sought to investigate the role of fibrin glue (Tisseel manufactured by Baxter India Pvt Ltd, Chennai, India) to reduce the chances of fistula formation in cases in proximal penile hypospadias. METHOD: A total of 120 patients with proximal penile hypospadias (patients having urethral meatus at posterior third of penile shaft and at penoscrotal junction) were included in the present study. Patients were randomly allocated into 2 groups of 60 each by using Strata 9 software random number table. In group A, fibrin glue was used as a sealant after hypospadias surgery, whereas in group B, no sealant was used. All the operations were performed by single surgeon using transverse preputial tubularized island flap urethroplasty. RESULT: Fistula formation occurred in 6 cases in group A (10%) and 19 cases in group B (32%) (P = .027). The fistulae observed in fibrin glue group A were single and small in size (<1 mm). Multiple (>or=2 fistulae) and larger fistulae (>2 mm) were observed in group B. Overall complication was significantly higher in group B (P = .006). CONCLUSION: Fibrin glue in hypospadias repair does not eliminate fistula formation. However, it seems that it minimizes the incidence of fistula formation.

A pilot study on the role of T-tube in typhoid ileal perforation in children.

BACKGROUND: Ileostomy is usually performed for patients of typhoid intestinal perforation with poor general condition, but it is associated with significant morbidity. We have used the T-tube in such patients as an alternative to ileostomy. METHODS: This is a prospective evaluation of a cohort of children with proven typhoid intestinal perforation. Patients with multiple perforations and poor general condition were managed with a T-tube inserted into the bowel lumen after closing all distal perforations (group 3). They were compared with patients who had primary closure of perforation (group 1) or bowel resection (group 2) to determine the efficacy of the use of T-tube. RESULTS: The total number of patients for groups 1, 2, and 3 was 51, 4, and 12 (n = 67). The mean number of perforations for the three groups was 1, 3.5 +/- 0.58, and 4.25 +/- 0.97. The operation time for the three groups was 37.29 +/- 3.24, 59.25 +/- 3.09, and 59.17 +/- 4.17 minutes, respectively. The T-tube was removed after 13.17 days. The mean duration of fistula at T-tube site to heal was 8.58 +/- 2.11 days. The overall follow-up period was 10.94 +/- 1.15 months and none of the patients with T-tube placement had features of intestinal obstruction. CONCLUSIONS: In children with multiple typhoid intestinal perforations and poor general condition, the use of T-tube may be an effective management option.

Is pneumoperitoneum an absolute indication for surgery in necrotizing enterocolitis?

BACKGROUND: Necrotizing enterocolitis (NEC) is the most common gastrointestinal medical/surgical emergency in neonates. Non-operative support is needed in 70% of NEC cases, and surgical intervention in the rest 30%. Historically, pneumoperitoneum has been considered as an absolute indication for laparotomy. In the present study we emphasize that pneumoperitoneum is not an absolute indication for exploratory laparotomy in NEC cases. METHODS: We prospectively studied 58 patients with severe NEC having pneumoperitoneum on abdominal X-ray in the last 5 years. At the time of admission, the patients were given intravenous fluid, total parental nutrition, blood transfusion and broad spectrum antibiotics followed by abdominal tapping (paracentesis). All the patients with pneumoperitoneum were closely monitored for 48 hours if abdominal tapping was repeated. When the disease seemed to worsen clinically, radiologically and laboratorially, the patient was subjected to exploratory laparotomy. RESULTS: Of the 58 patients, 40 were treated conservatively whereas 18 underwent surgical intervention. The overall mortality in the present study was 12.1%, including 5% of the patients managed conservatively and 27% of the patients undergoing surgery. CONCLUSIONS: Pneumoperitoneum is not an absolute indication for surgery in cases of neonatal NEC. Most of the patients can be treated conservatively.

Juvenile polyposis syndrome.

AIM: Report of a series of 12 cases of juvenile polyposis coli. METHODS: The study period was from 1995 to 2005. All the patients were treated by total colectomy with rectal mucosectomy and endorectal ileoanal pullthrough with or without ileal pouch formation. Covering ileostomy was avoided in all the cases. Time taken for the surgery, postoperative complications and continence were documented. RESULTS: The mean operating time was 4.2 h (range: 4-5 h). The mean duration of hospital stay was 16.3 days (range: 15-18 days). The most common postoperative complication was pouchitis and perianal excoriation. Initially, all the patients were passing stools at an interval of 2 h, and after 3 weeks, the frequency has reduced to 6-8 stools per day. In the follow-up after 3 months, the frequency was 3-5 per day with minimal soiling. CONCLUSIONS: Single-stage total colectomy with rectal mucosectomy and endorectal ileoanal pull-through without covering ileostomy and pouch formation is a safe and definitive treatment for juvenile polyposis coli if the patient selection is appropriate.

Role of fibrin glue as a sealant to esophageal anastomosis in cases of congenital esophageal atresia with tracheoesophageal fistula.

OBJECTIVE: The aim of this study was to characterize a successful approach for the management of infants with long-gap esophageal atresia (EA) with tracheoesophageal fistula (TEF). The goal was to preserve the native esophagus and minimize the incidence of esophageal anastomotic leaks using fibrin glue as a sealant over the esophageal anastomosis. METHOD: A total of 52 patients were evaluated in this study. Only patients in whom, gap between the two ends of the esophagus was > or = 2 cm were selected during January 2005 to January 2007. Patients were divided in two groups on the basis of block randomization. Group A comprised the patients in whom fibrin sealant was used as reinforcement on a primary end-to-end esophageal anastomosis; in group B, fibrin glue was not used. The two groups were compared in terms of esophageal anastomotic leak (EL), postoperative esophageal stricture (ES), and mortality. The statistical analysis was done using Fisher's exact test and the chi-squared test. RESULT: The number of anastomotic leaks in group A (glue group) was about one-fifth that in group B (no glue group). The incidence of ES was almost twice as high in group B as in group A. The mortality rate was almost threefold higher in group B (no-glue group). The higher incidence of EL and ES in group B compared to group A was statistically significant. CONCLUSION: Thus, fibrin glue when used as an adjunct to esophageal anastomosis for primary repair of long-gap EA with TEF appears safe in the clinical setting and may lower the chances of esophageal leak and anastomosis-site strictures. Hence, it can diminish the mortality and morbidity of these patients.

Conservative treatment for round worm intestinal obstruction.

OBJECTIVE: The ascariasis is one of the most cosmopolitan intestinal parasite infections and it can be in inhospitable regions inhabited by human being, but its biggest prevalence is observed in the tropical and subtropical areas. Intestinal obstruction has been estimated to occur in 2 per 1000 ascaris-infected children per year. We are presenting a study emphasizing the conservative treatment for complete intestinal obstruction due to roundworms without sign and symptom of peritonitis and perforation. METHODS: A total of 22 patients of roundworm obstruction partial or complete without signs of and symptoms of peritonitis were admitted in the Department of Pediatric Surgery IMS, BHU Varanasi India in the period form 2003-2005. Patients were put nil by mouth, intravenous fluid, antibiotics, piperazine salt through nasogastric tube and glycerine + liquid paraffin emulsion enemas and were evaluated for duration of hospital stay, rate of conversion to surgical treatment and complications. RESULTS: 19 (86%) patients were treated successfully with conservative line of management. Only 3 patients required surgical intervention. No mortality, complication and mean hospital stay was 4.1 days (range 4-5 days). CONCLUSION: Round worm intestinal obstruction can be effectively treated by conservative line of management.

Obstructed Morgagni's hernia.

Morgagni's Hernia is the direct herniation of the intra-abdominal organs through the anterior retrocostoxiphoid diaphragmatic defect. We are presenting a case of obstructed Morgagni's hernia in a five-year-old child. Who presented with colicky abdominal pain, distention of upper abdomen, vomiting with constipation of three days duration.. Morgagni hernia is a rare disease even rarer in pediatric age group, and is usually diagnosed incidentally; presentation of this disease with obstruction had been reported in adult but not in children.