Inflammatory Myofibroblastic Tumour in Children: A Report of Two Cases and Review of Literature.

ABSTRACT: Inflammatory myofibroblastic tumour in paediatric patients present with a diagnostic dilemma because of its clinical, radiological and histopathological features overlapping with other mesenchymal tumours common in this age. Because of its rarity, the exact features are still unclear. Here, we are reporting clinical, radiological and histopathological appearances of two such cases. In both cases, the exact diagnosis was confirmed only after immunohistochemistry. There is a need for further detailed study to exactly determine the natural course and prognosis of these tumours.

Gastric Teratoma in Children: A Report of Two Cases and Review of Literature.

ABSTRACT: Gastric teratomas are very rare tumours. They present with upper abdomen distension which can easily be confused with other common conditions with mass per abdomen in the paediatric age group. Surgical excision is curative for gastric teratomas. The diagnosis is confirmed by radiological evaluation followed by histopathological analysis of excised specimen. We are reporting two such cases of gastric teratomas who presented with complaints of upper abdomen distension. They were evaluated with radiological imaging and successfully managed by surgical excision.

Congenital cardiac anomalies in non-syndromic cleft lip and cleft palate patients: A systematic review and meta-analysis.

The aim was to establish a specific and definite connection between non-syndromic orofacial cleft patients and associated congenital heart disease (CHD). Following PRISMA guidelines, selective databases were searched for data collection. Studies showing a definite association of CHD with orofacial cleft were included, and studies non-specific of the association of orofacial cleft with CHD were excluded. Data extraction criteria were study design, frequency of CHD in overall non-syndromic orofacial cleft and in specific cleft type, and most prevalent congenital cardiac anomaly. DerSimonian Laird random effects model was used to estimate the pooled proportion of CHD, along with corresponding 95% confidence intervals (CIs) for each measure. Publication bias was assessed using Fail-Safe N analysis and the Rosenthel approach. Of a total of 182 articles searched, only 30 studies were assessed. The overall pooled estimate of the proportion of CHD in total cleft lips/palates was 16% (95% CI: 13-19). The odds of developing CHD in cleft palates was 4.08 times more as compared to cleft lips with 95% CIs of 3.86-4.33, and 1.65 more as compared to cleft lips and palates both with 95% CI of 1.52-1.68. We affirm the upsurging prevalence of CHD in non-syndromic cleft children and vehemently propose that it is of utmost importance to inculcate it in practice and policy-making to screen all non-syndromic orofacial cleft children for congenital cardiac anomaly. This study was registered on PROSPERO (ID no. CRD42023391597) on February 24, 2023.

Advocating for Routine Post-natal Surveillance and Urgent Neonatal Inguinal Hernia Repair.

ABSTRACT: Inguinal hernias are more common in preterm and neonates and incidence of incarceration are reported to be more in the first 6 months of life. Strangulation follows incarceration and various incarcerated and strangulated contents having been reported in the sac. The fistulation of the herniated content through the scrotal skin is quite rare with only a few reported cases. We present the case of entero-scrotal fistula in a neonate managed with staged repair along with a brief review of the literature. A 27-days-old, full-term male presented with faecal discharge from the right scrotum. He had no tell-tale signs of obstructed hernia. A faecal fistulous opening was located in the right hemi-scrotum. The terminal ileum was seen as the content with an antimesenteric perforation, divided ileostomy and scrotal debridement was done and later ileo-ascending anastomosis was performed electively. Neonatal hernias should be considered an urgency and we advocate early surgery. The resource-limited setting and poor post-natal surveillance may have added to the worries. In our case, swelling and fistulation occurred in a very brief period of 36 h. We managed the child with a diversion stoma followed by ileo-ascending anastomosis later.

Giant Colpolithiasis in Urogenital Sinus Anomaly with Urethral Duplication: A Case Report and Review of Literature.

ABSTRACT: Primary vaginal calculi are uncommon in children. Urethral duplication in females is seen to occur in association with complex congenital malformations. We report the case of perianal persistent urogenital sinus with a hypertrophied clitoris with phallic urethra, scrotum-like pouch, uterus didelphys with obstructed hemivagina, and giant colpolithiasis in 46XX female. A 16-year-old presented with pain abdomen and cyclic passage of blood clots per rectum. She had a tender lump in left iliac region, a phallus like protrusion and a ruggous sac below it. Vaginal opening was absent. Computed tomography showed two uterine horns with a separate cervix and distended non-communicating hemivaginas with a large calcified oval mass in the left hemivagina. On exploration, calculus was extracted from the left hemivagina. The large calculus found in the left hemivagina appears to be the cause of all presenting symptoms. It obstructed the left hemivagina, filling the left uterine horn with menstrual blood causing its gradual enlargement and secondary infection. The early diagnosis and prompt referral of such an anomaly can only be ensured in institutional deliveries. For a significant proportion of newborns in the developing world, the ability to afford or even be referred to institutes which deal with such cases is a luxurious affair. We hope to bridge bridging the knowledge, attitude and practice gap that exists in our health-care system with this report.

Congenital Urethrocutaneous Fistula.

ABSTRACT: Male congenital urethrocutaneous fistula is an extremely rare anomaly that is commonly associated with chordee or anorectal malformations. It is characterised by an abnormal urethral opening on the ventral aspect of the penis with well-formed distal urethra and meatus at the tip of the glans. The treatment is individualised according to the site of the fistula, associated anomalies and condition of the distal urethra. The principles of hypospadias surgery should be strictly followed.

Neonatal Sigmoid Colon Perforation in Low Anorectal Malformation.

Anorectal malformations (ARMs) are common congenital anomalies in neonates. Colonic perforation is very rare in ARMs. Delay in diagnosis of neonate with ARM results in colonic perforations and life-threatening morbidity. Colonic perforation due to ARM may not be completely avoided; however, early diagnosis and management are essential in assuring better outcomes.

Paediatric laparoscopic inguinal hernia repair: Level of disconnection of peritoneal cuff - An observational study.

Introduction: Inguinal hernia is a common surgical condition in children. Conventionally, the open approach for inguinal hernia repair has been considered the gold standard. However, in the past two decades, laparoscopic inguinal hernia repair has gained popularity among paediatric surgeons as an alternative to the open approach. Apart from good cosmesis and shorter stay at hospital, laparoscopy offers clear-cut advantages of visualising contralateral site and simultaneous repair if it is patent. Many techniques for laparoscopic inguinal hernia repair have been proposed. In this retrospective observational study, we are comparing outcomes between proximal and distal disconnection of hernia sac. Materials and Methods: Ninety-five patients with inguinal hernia were studied in two groups. Group A included 50 patients in which hernia sac was disconnected from the peritoneal cuff proximal to deep inguinal ring (DIR). Group B included 45 patients in which hernia sac was disconnected distal to DIR. Various sociodemographic parameters and intraoperative findings were compared. Outcomes were analysed in terms of post-operative pain, duration of stay at the hospital and recurrences. Results: In group A, there were 46 males and four females with mean age of 4.01 years with standard deviation (SD) of 2.96. Group B included 37 males and eight females with mean age of 5.09 years with SD of 3.56. Excess post-operative pain was observed in 33 patients in Group A with proximal disconnection of hernia sac whereas it was seen in only three patients in Group B with distal disconnection of sac. The P was 0.001 which was highly significant. The duration of stay in the hospital was more in Group A (2.36 ± 1.22 days) as compared to Group B (1.8 ± 0.66 days) with a P of 0.0076 which was significant. Hernia recurrence was seen in four out of 50 patients in Group A (8%) as compared to no recurrence in Group B. However, the difference was not significant. Conclusion: The disconnection of hernia sac distal to DIR is associated with less post-operative pain and shorter duration of hospital stay. There is less recurrence seen in distal disconnection of hernia sac as compared to proximal disconnection; however, to achieve the level of significance, a large cohort study is required.

Long-term complications after extrahepatic cyst excision for type IV-A choledochal cysts.

Forty-five adults with type IV-A choledochal cysts (CDC) who underwent extrahepatic cyst excision from January 2013 to December 2021 were followed up for a median interval of 25 months (range, 2 to 10 years) to observe the long-term complications in the remaining intrahepatic cyst. Late complications in varying combinations were seen in 10 patients, which included cholangitis and/or intrahepatic stones in 9 patients, intrahepatic bile duct stenosis with stones in 2 patients, anastomotic stricture in 6 patients, and left lobar atrophy with intrahepatic stones in 3 patients. Out of 6 patients who required re-do hepaticojejunostomy (HJ), three patients had left lobe atrophy with patent HJ anastomosis and a recurrent attack of cholangitis on follow-up at 3, 8, and 10 years. Complications occur frequently after extrahepatic cyst excision for type IV-A CDC and require a long-term follow-up.

Perioperative role of oral gabapentin as an analgesic in paediatric patients: A randomised controlled trial.

Background and Aims: Surgical procedure commonly performed in the advanced pediatric age group includes urogenital surgery, adenotonsillectomy, etc., Aim: The aim of this study is to determine the effect of single-dose gabapentin 15 mg/kg on acute pain in the immediate postoperative period in patients aged 8-14 years undergoing surgeries under general anesthesia. Material and Methods: After the approval from the institutional ethical committee, 60 American Society of Anesthesiologists (ASA) I and II patients aged 8-14 years undergoing urogenital surgeries (orchidopexy/urethroplasty) under general anesthesia were included in this study. The patients were assigned into one of the two treatment groups. Patients in group I received oral gabapentin 15 mg/kg dissolved in 5 mL of honey 2 h before surgery, while patients in group II received 5 mL honey orally 2 h before surgery. Results: A total of 60 patients participated. Patients in group I had lower consumption of fentanyl perioperatively (intraoperatively: 1.36 ± 0.70 mcg/kg; postoperatively: 2.36 ± 0.795 mcg/kg) than group II (intraoperatively: 1.8 ± 0.6 mcg/kg; postoperatively: 2.9 ± 0.47 mcg/kg). The differences in the two groups were significant. The time to first rescue analgesia was greater in group I (3.03 ± 0.60 h) than in group II (2.26 ± 0.57 h). There was an increase in sedation score in the treatment group. Conclusion: Our clinical study demonstrates that a 15 mg/kg single preemptive oral dose of gabapentin might reduce the requirement of analgesics perioperatively in pediatric urogenital surgery but might also be associated with undesirable effects such as increased sedation.

Internal fat prolapse from ischiorectal fossa masquerading as currarino syndrome.

Pelvic magnetic resonance imaging (MRI) is the latest addition to already existing tools for evaluation of anorectal malformation (ARM). It provides detailed information about pelvic floor musculature as well as position of rectal pouch. However, lack of knowledge about normal pelvic floor anatomy can lead to misinterpretation of pelvic MRI which can create confusion and change in approach for surgery. A 14-month-old male child with diagnosis of ARM was evaluated with pelvic MRI. There was finding of abnormal protrusion of fat through the ischiorectal fossa which was misinterpreted as Currarino syndrome which created confusion immediately before posterior sagittal anorectoplasty. Pelvic MRI is highly informative while evaluating a case of ARM. However, a detailed knowledge of pelvic floor anatomy is mandatory to avoid wrong interpretation and misdiagnosis.

Comparison of the oropharyngeal leak pressure between three second generation supraglottic airway devices during laparoscopic surgery in pediatric patients.

BACKGROUND: Previous studies have shown Proseal LMA and I gel similar to endotracheal intubation in ventilatory ability in pediatric laparoscopic surgeries. AIMS: The primary aim of this study was to assess whether there is a significant difference in the oropharyngeal leak pressure between Ambu Auragrain, I-gel, and Proseal LMA during pediatric laparoscopic surgery. METHODS: In this randomized controlled trial, 90 male patients of American Society of Anesthesiologists physical status I aged between 6 months and 10 years who were scheduled for laparoscopic single-sided inguinal hernia repair were recruited and randomly allocated to three groups in which airway was secured with Ambu Auragain, I gel, or Proseal LMA. The primary outcome was oropharyngeal leak pressure. The secondary outcomes were peak pressures before and after pneumoperitoneum, fiberoptic view, insertion attempts, insertion time, manipulations, perioperative and postoperative anesthesia-related problems. Continuous variables were compared using the one-way analysis of variance or the Kruskal-Wallis test with post hoc Turkey analysis. Categorical and ordinal data were compared using the chi-square test or Fisher's exact test. RESULTS: Oropharyngeal leak pressure before pneumoperitoneum was higher with I gel as compared to Ambu Auragain (27.36 ± 5.72 cm of H2 O vs 23.56 ± 5.72 cm of H2 O) (p = .021) and PLMA (27.36 ± 5.72 cm of H2 O vs 23.24 ± 4.35 cm of H2 O) (p = .011). Oropharyngeal leak pressure after pneumoperitoneum was also higher with I gel as compared to Ambu Auragain (31.58 ± 4.35 cm of H2 O vs 26.83 ± 5.00 cm of H2 O) (p = .001) and Proseal LMA (31.58 ± 4.35 cm of H2 O vs 27.03 ± 3.80 cm of H2 O) (p = .002). Oropharyngeal leak pressures of Ambu Auragain and Proseal LMA were comparable. Postoperative complications were similar in all the supraglottic airway devices. No regurgitation or aspiration-related problem was observed in our study. CONCLUSION: I gel had a higher oropharyngeal leak pressure than the other two supraglottic airway devices and therefore may represent a better choice in situations where higher ventilatory pressures may be necessary, for example, in extremes of weight trendelenburg position, etc. CLINICAL TRIAL IDENTIFIER: Clinical trial registry of India (CTRI/2018/11/016445).

Colonic Agenesis with Anorectal Malformation - Rarest Variant of Congenital Short Colon.

We report a case of colonic agenesis with anorectal malformation in a newborn girl. The patient also presented with congenital heart disease. We are presenting the clinical features, intraoperative findings, and treatment plan.

Profile and Clinical Outcome of Children with Wilms' Tumor treated at a Tertiary Care Centre, India.

Pritanjali SinghBackground Wilms' tumor (WT) is the most common kidney tumor of the pediatric age group. The outcome of WT has improved due to the evolution of the treatment approach. A prospective observational study was conducted at All India Institute of Medical Sciences (AIIMS), Patna, to analyze the clinical profile along with the response and outcome to neoadjuvant chemotherapy according to the International Society of Pediatric Oncology (SIOP) protocol. Materials and Methods In total, 28 patients of WT visited the radiotherapy department from January 2015 to December 2019. Results Gender distribution showed male preponderance with a median age at diagnosis was 31 months. The abdominal lump was the dominant clinical presentation. The median volume of tumor at diagnosis was 359.48 mL (52.67-1805.76). Radiological staging workup shows that stage I, II, III, IV, and V were 7.1%, 39.3%, 39.3%, 10.7%, and 3.6% respectively. Neoadjuvant chemotherapy (NACT) was received by all patients. Also, 71.4% of patients showed > 50% of tumor volume reduction, while 28.6% of patients showed < 50% of tumor mass reduction. There was a statistically significant decrease in the tumor volume reduction following neoadjuvant chemotherapy ( p < 0.001). There was a statistically significant stage down ( p = 0.018) of the disease. Bivariate correlation studies showed recurrence was correlating statistically significantly with age < 24 months ( p = 0.049), locoregional lymph nodes ( p = 0.008), histopathological subtypes ( p < 0.001), stage of the disease ( p = 0.003), and risk groups ( p < 0.001). In addition, 25% of patients developed recurrence during the median follow-up of 25 months. The median disease-free survival (DFS) and overall survival (OS) were not reached. The mean DFS and OS were 48 and 59.13 months, respectively. One- and 3-year DFS were 100% and 64.1%, respectively. One- and 3-year OS were 100% and 75% respectively. Conclusion Our study suggests that most of the patients presented at an advanced stage, thus rendering most of the cases difficult to undergo surgery at presentation. Neoadjuvant chemotherapy followed by surgery may be considered a well-balanced approach with a comparable response and survival outcomes.

Analysis of outcomes of biliary reconstruction after post-cholecystectomy bile duct injuries.

BACKGROUND: Post-cholecystectomy bile duct injury (BDI) is a serious complication that often requires surgical repair. This study aimed to analyze the outcomes of surgery performed for BDI and to determine the factors associated with post-surgical complications. METHODS: A retrospective analysis was conducted using a prospectively maintained database of 105 patients who underwent surgical repair for post-cholecystectomy BDI between March 2013 and March 2020. BDI was classified based on the Strasberg-Bismuth system, and the outcomes were graded using the McDonald criteria. Multivariable logistic regression was used to identify the significant variables associated with postoperative complications. RESULTS: In a cohort of 105 patients with post-cholecystectomy BDI who underwent bilioenteric repair, 71 (67.6%), 25 (23.8%), 2 (1.9%), and 7 (6.7%) patients had excellent, good, fair, and poor outcomes, respectively, during a median follow-up of 64 months. The incidence of recurrent biliary stricture after definitive surgical hepaticojejunostomy was 6.7% (n = 7). The presence of cholangitis, choledochoduodenal fistula, and hilar biliary strictures was among the significant variables associated with the development of both short-term and long-term complications following surgery. CONCLUSIONS: Surgical repair of BDIs with bilioenteric anastomosis can yield excellent results when managed in a tertiary care center where expertise in the reconstruction of the biliary tree is prioritized.

Clinical profile and prognostic factors of alcoholic cardiomyopathy in tribal and non-tribal population.

OBJECTIVE: Alcoholic cardiomyopathy (ACM) is a leading cause of non-ischaemic dilated cardiomyopathy (DCM) in tribal and non-tribal population. However, no study has been done depicting the correlation between clinical profile and prognosis of ACM in tribal and non-tribal population. This study also defines the long-term outcome and prognostic markers of ACM. METHODS: We studied 290 patients with ACM who were evaluated in our institute between January 2013 and December 2016. The primary endpoint of the study was all-cause mortality. Statistical analysis was done by using Kaplan-Meier survival curves for the assessment of all-cause mortality and Cox regression for the assessment of risk factors. RESULTS: After a median follow-up period of 3.75 years (IQR: 3-4 years), 50 patients with ACM (37.3%) died among tribal population while 14 patients (9%) died among non-tribal population. Independent predictors of all-cause mortality in ACM identified by Cox regression were left ventricular ejection fraction (LVEF) (HR: 0.883; 95% CI 0.783 to 0.996; p=0.043), QRS duration (HR: 1.010; 95% CI 1.007 to 1.017; p=0.005) and Child-Turcotte-Pugh (CTP) Scoring (HR: 12.332; 95% CI 6.999 to 21.728; p<0.001) at admission. The Kaplan-Meier survival probability estimate was 95.1% at 1 year and all-cause mortality was found to be higher in patients with QRS>120 ms, LVEF ≤35%, CTP Grade B/C than patients with QRS≤120 ms, LVEF >35% and CTP Score A, respectively (log-rank χ²=55.088, p<0.001; log-rank χ²=32.953, p<0.001; log-rank χ²=139.764, p<0.001, respectively). CONCLUSION: Our study indicated increased morbidity and mortality in tribal population. LVEF, QRS duration and CTP Scoring at the time of presentation were found to be the independent prognostic markers of patients with ACM.