A Novel Association of Colonic Duplication with Mayer-Rokitansky-Kuster-Hauser Syndrome in an Adolescent Girl.

The association is defined as two or more malformations with unclear relationships, which do not fit the criteria of a syndrome. This case report presents a rare finding of tubular colonic duplication in association with Mayer-Rokitansky-Küster-Hauser syndrome in an adolescent girl. The patient presented with gastrointestinal bleeding and abdominal pain, necessitating surgical intervention. The successful excision of the duplicated segment highlights the importance of prompt diagnosis and treatment in such cases.

A Pilot Survey of Indian Stakeholders: Parents, Doctors, and Grown-Up Patients of Disorders of Sexual Differentiation on Management Decisions and Associated Gender Dysphoria.

Background and Aims: Of late, there are many legal representations from select quarters to halt all medical interventions in children with differences of sex development (DSD). In this survey on management decisions in DSD, we distil the views of Indian stakeholders: parents, physicians, and grown-up patients with DSD on their management decisions to identify decisional satisfaction or gender dysphoria. Methods: The survey domains included the patient demographics, final diagnosis, decision on the sex of rearing, surgical interventions, opinion of the stakeholders on the preferred age of sex assignment, final sex of rearing, and agreement/disagreement about sex assignment (gender dysphoria). Results: A total of 106 responses were recorded (66% parents, 34% grown-up patients aged 12-50 years). Among parents, 65/70 (95%) preferred the sex to be assigned soon after birth. All grown-up patients preferred sex to be assigned soon after birth. Regarding decisions on surgery, 74% of physicians and 75% of the grown-up patients felt parents should be allowed to decide interventions. Among Indian parents, 90% felt they should have the right to decide surgery in the best interest of their child for a safe social upbringing. Overall, gender dysphoria among Indian DSD patients was <1% (1/103, 0.97%). Conclusions: The predominant preference and opinion of major Indian stakeholders (physicians, parents, and grown-up DSD patients) support the existing approach toward DSD management, including early sex assignment and necessary medical intervention.

Role of Urinary Biomarkers (Transforming Growth Factor ß1, Neutrophil Gelatinase-Associated Lipocalin, and Cystatin C) as a Prognostic Factor of Renal Outcome in the Posterior Urethral Valve.

Background: The urinary biomarker response precedes the appearance of any renal structural or functional derangement. Transforming growth factor-ß1 (TGF-ß1), neutrophil gelatinase associated lipocalin (NGAL), and Cystatin C (CysC) can act as the early prognostic markers in posterior urethral valve (PUV) patients. Aim: To compare the urinary levels of TGF-ß1, NGAL, and CysC between PUV cases and age matched controls and to correlate these with renal structural and functional parameters. Materials and Methods: This prospective study included children with PUV diagnosed using the standard investigations and an equal number of age-matched controls with nonurological problems. For the study subjects, the urinary samples were collected at three different time points (pre- and postoperatively at 3 and 6 months), whereas for controls, only single-voided samples were studied. The urinary levels of TGF-ß1, NGAL, and CysC were estimated by the standardized techniques using the ELISA kits. Statistical methods were used to drive the comparisons between cases and controls. Results: Fifteen children with a median age of 10 (5-48) months were enrolled in each of the two groups. The mean uTGF-ß1 in the case group was significantly higher at all three time points (43.20 ± 6.13 pg/ml, 43.33 ± 11.89 pg/ml and 40.71 ± 9.01 pg/ml) as compared to the control group (29.12 ± 8.31 pg/ml) (P ≤ 0.001). The median uNGAL in the case group was also higher (17.78 ng/ml, 2.35 ng/ml and 2.536 ng/ml) as compared to the control group (1.31 ng/ml). However, the difference was significant only preoperatively (P = 0.02). The median uCysC in case group was similarly higher (0.347 µg/ml, 0.439 µg/ml, and 0.382 µg/ml) than the control group (0.243 µg/ml) (P > 0.05). Serum creatinine in the case group (0.49 mg/dl) showed no significant rise above that of control (0.24 mg/dl). A cutoff value of uTGF-ß1 = 36.55 pg/ml (P < 0.001), uNGAL = 0.879 ng/ml (P = 0.02), and uCysC = 0.25 µg/ml (P = 0.22) was found to be associated with renal damage in PUV. A significant correlation was found between uNGAL and S. creatinine at 3 months (r = 0.43, P = 0.017) and 6 months (r = 0.47, P = 0.08). Conclusion: The elevated uTGF-ß1, a decline in uNGAL and an increase in uCysC suggests ongoing inflammation, improvement in hydronephrosis and a prolonged proximal tubular dysfunction in PUV patients, respectively.

Ileal Trichobezoar Presenting with Acute Appendicitis and Intestinal Obstruction: A Rare Case Report and Review of Literature.

Trichobezoar is a rare diagnosis among pediatric patients highlighting underlying psychiatric illness. Gastric bezoar with a long tail extending into small bowel may present with varied presentation including small bowel obstruction. Isolated small bowel trichobezoar is rare making diagnosis difficult highlighted in the index case.

Foreign Body Ingestion in Children: An Experience of 99 Cases in a Tertiary Care Center in Delhi.

Background: Accidental ingestion of foreign bodies in children is critical, as the inability to effectively communicate can potentially lead to devastating consequences. We aimed to determine the epidemiology of foreign body ingestion and variability according to age, gender, type, and location of foreign body, and describe its management. Aim and Objective: The aim was to study the various types of foreign body ingestions in children admitted to pediatric surgery and their management. Materials and Methods: A retrospective study was conducted from January 2020 to June 2022 on children under the age of 12 years with a confirmed diagnosis of foreign body ingestion. Patients were clinically and radiologically assessed, after which standard protocols were followed wherein patients were followed by either observation or emergent management. Emergent management included removal of the foreign body by either endoscopy or surgery. Comparisons among multiple age groups, gender, type of foreign body, location of foreign body, and their management were analyzed. Results: Out of 99 subjects in our study, there were 76 boys and 23 girls. The median age of presentation was 5 years. Most children were asymptomatic at presentation. The most frequently ingested foreign body was a coin in all age groups. The majority of the foreign bodies were suspected to be in the small bowel. The foreign bodies that had crossed the duodenojejunal flexure (n = 74, 74.7%) were managed conservatively with the observation of a variable period of a minimum of 24 h and a maximum of 48 h. 21 cases were managed by endoscopic removal, while three cases required surgical intervention. Conclusions: Overall, the most common gastrointestinal foreign body was a coin in all age groups. Button battery is the most worrisome foreign body; however, depending on its position, it can be managed conservatively. Upper GI foreign bodies can be safely removed endoscopically. Parental counseling is very important for the prevention of ingestion of foreign bodies.

"Necklace of magnets" in the abdomen: an unusual case with "multiple" bowel perforations.

Magnetic foreign body ingestion poses a threat especially if more than one is ingested. If consumed alone, small magnetic foreign bodies are likely to pass without significant event; however, when multiple magnets are ingested, they can be attracted to each other through the intestinal wall, which may lead to serious consequences and complications, including bowel perforation, obstruction, peritonitis, and death. We report a case of a 2-years male child patient presented with multiple small round magnetic beads ingestion from a magnetic pendant that appeared like a necklace pearl after conglomeration on abdominal radiograph. On exploration, we found multiple perforations involving ileum, cecum, and transverse colon, with multiple conglomerated beads extruding from the perforation sites.

Single dartos flap versus double dartos flap in hypospadias repair: A systematic review and meta-analysis with trial sequential analysis and fragility index.

INTRODUCTION: Hypospadias is a common congenital urogenital anomaly. Despite advancements in surgical techniques, still it presents challenges in management. An important aspect of hypospadias repair is the use of protective layers to cover neourethra. This review focuses on comparing the Single Dartos Flap (SDF) and Double Dartos Flap (DDF) techniques, used to cover the neourethra. These techniques differ in terms of the number of dartos layers used to cover the neourethra. METHODS: This systematic review, follows PRISMA guidelines, included six RCTs from PubMed/MEDLINE, Cochrane Library, Scopus, Web of Science, and CINAHL. Patients with hypospadias repair with use of SDF or DDF were analyzed for outcome, Urethrocutaneous fistula, meatal stenosis, glans dehiscence, penile torsion and cosmetic outcomes. Statistical analysis was done using Review Manager, with TSA and FI ensuring result robustness. RESULTS: Six studies met inclusion criteria, and risk of bias assessment indicated low risk across all domains. Meta-analysis results favored DDF over SDF for reducing urethrocutaneous fistula (RR 0.37, 95% CI 0.20-0.68) but showed no significant difference in meatal stenosis and glans dehiscence. DDF also associated with lower risk of penile torsion (RR 0.05, 95% CI 0.01-0.35). CONCLUSION: The systematic review, based on randomized controlled trials (RCTs), provides evidence supporting the use of DDF over SDF in hypospadias repair, particularly in distal hypospadias using the TIP procedure. The article emphasizes the potential advantages of DDF in reducing UCF but further robust evidence is needed to confirm these results based on the findings of TSA and FI.

Pediatric Age-adjusted Shock Index (SIPA): From Injury to Outcome in Blunt Abdominal Trauma.

Purpose: The Shock Index Pediatric Age-Adjusted (SIPA) score is a useful tool for identifying pediatric trauma patients at a risk of poor outcomes and for triaging. We are studying the relationship between elevated SIPA score and specific outcomes in pediatric trauma patients. Materials and Methods: A retrospective study was conducted in which case records of 58 pediatric patients with blunt abdominal trauma were evaluated and tabulated for their SIPA scores only at the time of their initial presentation and categorized into two groups - normal SIPA and elevated SIPA. The primary outcomes were need for blood transfusion, need for any intervention, and need for emergency surgery, and the secondary outcomes were need for computed tomography (CT) scan, need for a ventilator, intensive care unit (ICU) stay, length of hospital stay, and mortality. Statistical methods were applied to find a relationship between elevated SIPA score and the primary and secondary outcomes. Results: An elevated SIPA score was noted in 27 (46%) patients. There was a significant relationship between elevated SIPA scores and patients needing blood transfusion (68.75%, n = 11) and length of hospital stay (10.48 ± 7.54 days). A significant relationship between elevated SIPA score and need for emergency surgery (54.54%, n = 6), need for a CT scan (56%, n = 14), and ICU stay (50%, n = 2) was not found. Conclusion: We have seen in our study that elevated SIPA scores at presentation are significantly related to need for blood transfusion and length of hospital stay. In more than half of the patients, elevated SIPA was associated with need for emergency surgery and requirement of CT scan, but it was statistically not significant. Therefore, assessment of this parameter can help in identifying such poor outcomes.

Variation in the Expression of Interstitial Cell of Cajal-like Cell (CD117) Across Congenital Pelvic-ureteric Junction Obstruction and its Renal Sonological and Functional Correlation: A Prospective Observational Study.

Aims and Objectives: This study aims to study the variation in the expression of CD117-positive interstitial cells of Cajal-like cells (ICC-LC) across the upper urinary tract region in children presenting with pelvic-ureteric junction obstruction (PUJO) and its association with renal functional and sonological parameters of patients. Materials and Methods: A prospective observational study was done on 20 children with congenital PUJO who underwent dismembered pyeloplasty. All children underwent renal sonography (anteroposterior pelvic diameter [APPD], pelvicalyceal ratio [P/C ratio], Mid polar renal parenchymal diameter [MPPD]) and functional imaging scan (LLEC scan or DTPA scan). Three specimens were taken intraoperatively from above PUJ, at the level of PUJ, and below PUJ. Those were examined immunohistochemically using CD117 to count ICC-LC using standard criteria. Variation in the expression of CD117-positive ICC-LC was correlated with the abovestated parameters. Results: The number of CD117-positive ICC-LC showed a continuous decreasing trend above downward. P/C ratio and APPD showed a parallel trend with ICC-LC distribution, whereas split renal function (SRF) showed an inverse relationship with the expression of ICC-LC. Children with lesser severity of obstruction (APPD <30 mm and SRF >40%) showed a uniform decreasing trend in the number of CD117-positive ICC-LC across PUJ. Children with more severe obstruction (APPD >30 mm and SRF <40%) showed a decrease in the expression of ICC-LC up to the level of PUJO followed by a sudden relatively increased expression of ICC-LC below the obstruction. Conclusion: The expression of ICC-LC shows a uniformly decreasing trend across obstruction when the severity of obstruction is less. Resurgence in the number of ICC-LC below PUJ in subjects with severe obstruction hints at the emergence of a new pacemaker area below severely blocked PUJ akin to that seen in complete heart block patients and deserves early attention.

Modified Pediatric Penile Perception Scale to Evaluate Cosmetic Outcome in Children With Hypospadias Repair.

OBJECTIVES: To compare the interpretation for cosmesis post-hypospadias repair by child, parents and surgeons using modified Pediatric Penile Perception Scale (PPPS). METHODS: This cross-sectional study involving 50 children (aged 2 to 17 years) with hypospadias was conducted at the pediatric surgery department of our public sector tertiary care hospital. Subjects were assessed 6 months after completion of all stages of hypospadias repair. Cosmetic assessment was done using modified PPPS. We clubbed together the variables 'meatus' and 'glans' as MG (meatus-glans) complex due to their extreme proximity (embedding), while cosmesis of phallus was considered independently. The modified PPPS scoring parameters included phallus, MG complex, shaft skin, and general appearance. Independent assessment by surgeon, patients and parents was compared, and analyzed using SAS 9.2 statistical software. Cosmetic results of single vs staged repair, and different repair types was compared. RESULTS: Assessment using modified PPPS showed that MG complex cosmesis and skin scarring were the most heeded parameters by all three categories of observers. PPPS by surgeons remained least affected by phallic cosmesis and that of the patient by the overall phallic appearance. Tubularized incised plate urethroplasty (TIPU) scored better on cosmesis. CONCLUSION: Phallic cosmesis should be considered an independent variable for assessing cosmetic outcome of hypospadias, apart from MG cosmesis.

A Rare Case of Extragonadal Mixed Germ Cell Tumor of the Sacrococcygeal Region with Mature Thymic Tissue in the Teratoma Component.

A rare extragonadal mixed germ cell tumor of the sacrococcygeal area presenting with mature thymic tissue in the teratomatous component, a rare finding and the first reported case of such an association.

Role of Ultrasonography and Inflammatory Markers in Predicting Complicated Appendicitis.

Aim: The aim is to compare the diagnostic accuracy of laboratory investigations and ultrasonography (USG) in distinguishing complicated appendicitis (C-AA) from uncomplicated appendicitis (UC-AA). Materials and Methods: Forty-six children who underwent appendicectomy at our center between November 2018 and July 2020 were included. Based on intraoperative findings, they were divided into two groups - complicated (perforated, gangrenous, or associated with fecal peritonitis; n = 18) and UC-AA (n = 28). USG findings and inflammatory markers were compared in both groups at admission. Results: At admission, the mean values for total leukocyte count (TLC) (16090.56 vs. 11739.29 per mm3), high sensitivity C-reactive protein (hsCRP) (35.8 vs. 31.62 mg/L), and procalcitonin (PCT) (3.83 vs. 1.41 ng/mL) were significantly higher in C-AA. Visualization of a blind tubular aperistaltic structure was the only sonographic sign showing statistical significance - significantly lower in C-AA (50% vs. 90%). Independent predictors of C-AA were - duration of symptoms >48 h (odds ratio [OR] 6.3), free fluid/loculated collection in right iliac fossa (OR 3.75), TLC >11000/mm3 (OR 3.6), hsCRP >35 mg/L (OR 6.0), PCT >0.6 ng/mL (OR 4.02), and nonvisualization of appendix on USG (OR 8.33). Biochemical factors were sensitive (89%) and specific (55%) in differentiating C-AA from UC-AA but the addition of sonological parameters significantly improved the specificity of predicting complicated AA to 61% (P = 0.0036). Conclusion: Combining laboratory data with sonological findings significantly improves the predictive value for differentiating C-AA from UC-AA and can help decide operative approach and prognosticating.

Triple Diversion Technique in Complete Duodenal Transaction Following Blunt Trauma Abdomen: A Time-Tested Method in a Very Rare Injury.

Duodenal injury following blunt abdominal trauma is extremely rare in children and many times, it has delayed presentation, leading to increased morbidity and mortality. A patient with complete duodenal transaction is a surgical challenge and management involves the time of presentation and extent of visceral damage. A 10-year-old boy was brought with features of bowel perforation after road traffic accident and underwent emergency laparotomy which revealed complete transaction of duodenum at D1 and D2 and pyloroduodenal junction extending toward lesser curvature. Primary closure of pyloroduodenal junction and D1-D2 was done with omental patch along with triple tube decompression (cholecystostomy, gastrostomy, and jejunostomy). The patient had an uneventful recovery. Primary closure of disturbed ends with triple diversion is a safe approach in young children with complete duodenal transaction in absence of gross peritoneal contamination and early presentation.

Minimally Jointed Symmetrical Pygopagus Twins - Successful Surgical Separation.

Conjoined pygopagus accounts for about 17% of all conjoined twins and commonly share the gluteal region, terminal spine, lower gastrointestinal, urological, and reproductive tracts. This makes their separation a very challenging task. We report herein pygopagus twins who had minimal fusion in coccygeal region and could be separated without a complicated procedure. The case is being reported to add to the literature and emphasize that simple fusion in conjoined twins can be relatively easy to manage.

Laparoscopic versus open appendicectomy for complicated appendicitis in children: A systematic review and meta-analysis.

BACKGROUND: Laparoscopic appendectomy (LA) is the preferred approach in uncomplicated appendicitis. However, in patients with complicated appendicitis (CA), the best approach is still unclear though laparoscopy is being increasingly preferred over open appendicectomy (OA) nowadays. AIM: To comprehensively review the current literature and compare the associations of LA and OA concerning various postoperative outcomes in order to determine the best approach for children with CA. METHODS: The PRISMA guidelines were adhered to and an electronic database search was extensively performed. Data analysis, including subgroup analysis of randomized-control trials, was performed using RevMan 5.3. Methodological and statistical heterogeneity, as well as publication bias of the included studies, were assessed. RESULTS: Four randomized controlled trials (266 LA versus 354 OA) and thirty-six case-controlled trials (2580 LA versus 3043 OA) were included in the analysis. Compared to OA, LA has a shorter length of stay, a lower rate of surgical site infection as well as a significantly lower overall complication rate. The rates of intraabdominal abscess formation, post-operative fever, pneumonia and ileus are similar in the two groups. So are the rates of readmissions and reoperations. LA was also shown to have a shorter time taken to oral intake and a lesser requirement of analgesics as well as intravenous antibiotics. Operative time for OA was found to be significantly shorter than that for LA. CONCLUSION: This meta-analysis objectively demonstrates that laparoscopy has a better overall complication profile compared to OA and should be the procedure of choice in children with complicated appendicitis.

Simultaneous Endoscopic Management of Urethral Duplication and Postposterior Sagittal Anorectoplasty Urethral Diverticulum in a Boy with Anorectal Malformation.

We report a case of urethral duplication with high anorectal malformation (ARM). A 12-year-old boy who had undergone transverse colostomy on day 2 of life and posterior sagittal anorectoplasty (PSARP) for ARM (rectobulbar fistula) at 8 years and also had urinary dribbling from a midscrotal opening in addition to normal voiding, presented with recurrent urinary tract infections and dysuria. Midscrotal dribbling had continued postoperatively. Colostomy had been closed 3 months after PSARP. Investigations revealed a large urethral diverticulum at the site of rectourethral fistula due to a segment of the gut being left behind during fistula closure. In addition, he had a sagittal urethral duplication originating at bulbar urethra and opening externally at midscrotum. The highlight of this report is successful endoscopic management of both the urethral duplication and the diverticulum.

Intussusception in children aged under two years in India: Retrospective surveillance at nineteen tertiary care hospitals.

OBJECTIVE: Intussusception has been linked with rotavirus vaccine (RVV) as a rare adverse reaction. In view of limited background data on intussusception in India and in preparation for RVV introduction, a surveillance network was established to document the epidemiology of intussusception cases in Indian children. METHODS: Intussusception in children 2-23 months were documented at 19 nationally representative sentinel hospitals through a retrospective surveillance for 69 months (July 2010 to March 2016). For each case clinical, hospital course, treatment and outcome data were collected. RESULTS: Among the 1588 intussusception cases, 54.5% were from South India and 66.3% were boys. The median age was 8 months (IQR 6, 12) with 34.6% aged 2-6 months. Seasonal variation with higher cases were documented during March-June period. The most common symptoms and signs were vomiting (63.4%), bloody stool (49.1%), abdominal pain (46.9%) and excessive crying (42.8%). The classical triad (vomiting, abdominal pain, and blood in stools) was observed in 25.6% cases. 96.4% cases were diagnosed by ultrasound with ileocolic location as the commonest (85.3%). Management was done by reduction (50.8%) and surgery (41.1%) and only 1% of the patients' died. 91.1% cases met Brighton criteria level 1 and 3.3% Level 2. Between 2010 and 2015, the case load and case ratio increased across all regions. CONCLUSION: Intussusception cases have occurred in children across all parts of the country, with low case fatality in the settings studied. The progressive rise cases could indicate an increasing awareness and availability of diagnostic facilities.

Comparative evaluation of I-gel vs. endotracheal intubation for adequacy of ventilation in pediatric patients undergoing laparoscopic surgeries.

BACKGROUND AND AIMS: The use of newer supraglottic devices has been extended to laparoscopic procedures. We conducted this study to compare and evaluate the efficacy of these two devices in pediatric laparoscopic surgeries. MATERIAL AND METHODS: Eighty children, 2-8 years of age, scheduled for elective short laparoscopic procedures were randomly allocated to the I-gel or endotracheal tube (ETT) group. Standard anesthesia protocol was followed for inhalational induction. I-gel or ETT was inserted according to the manufacturer's recommendations. Ventilation was set with tidal volume 10 ml/kg and a respiratory rate of 16/min. Carboperitoneum was achieved up to an intra-abdominal pressure of 12 mmHg. STATISTICAL ANALYSIS: The primary outcome variable was adequacy of ventilation (peak airway pressure, end-tidal CO2, minute ventilation, and SPO2). These variables were recorded after securing airway, after carboperitoneum and desufflation of the peritoneal cavity. The oropharyngeal leak pressures were also noted. Statistical analysis was done using SPSS software version 17.0. P <0.05 was considered statistically significant. RESULTS: No significant difference was observed in the heart rate or mean arterial pressure. There was a significant increase in the PECO2 and peak airway pressure after creation of carboperitoneum. There was significant increase in minute ventilation in both groups after creation of carboperitoneum. CONCLUSION: To conclude, I-gel is comparable to endotracheal intubation in terms of adequacy of ventilation. The increase in peak airway pressures is less with I-gel. In addition, postoperative complications are fewer with I-gel.

Formulation of Research Question - Stepwise Approach.

Formulation of research question (RQ) is an essentiality before starting any research. It aims to explore an existing uncertainty in an area of concern and points to a need for deliberate investigation. It is, therefore, pertinent to formulate a good RQ. The present paper aims to discuss the process of formulation of RQ with stepwise approach. The characteristics of good RQ are expressed by acronym "FINERMAPS" expanded as feasible, interesting, novel, ethical, relevant, manageable, appropriate, potential value, publishability, and systematic. A RQ can address different formats depending on the aspect to be evaluated. Based on this, there can be different types of RQ such as based on the existence of the phenomenon, description and classification, composition, relationship, comparative, and causality. To develop a RQ, one needs to begin by identifying the subject of interest and then do preliminary research on that subject. The researcher then defines what still needs to be known in that particular subject and assesses the implied questions. After narrowing the focus and scope of the research subject, researcher frames a RQ and then evaluates it. Thus, conception to formulation of RQ is very systematic process and has to be performed meticulously as research guided by such question can have wider impact in the field of social and health research by leading to formulation of policies for the benefit of larger population.