Background: Jejunoileal atresia (JIA) represents a common cause of neonatal bowel obstruction. There is a discrepancy between the diameters of the proximal and the distal bowel loops and this is managed with excision or tapering of the dilated proximal bowel loop. We aim primarily to evaluate the outcome of tapering enteroplasty (TE) in managing JIA and secondarily to compare the outcome of TE to non-TE. Materials and Methods: A retrospective analysis of records of all neonatal admissions with JIA from January 2017 to December 2018 at a tertiary university children's hospital. Type and location of atresia, time to full enteral feeds, length of stay (LOS), complications, and outcome were assessed in TE and non-TE groups. Results: Forty-one patients were included in the study; 29 (70.7%) cases had jejunal atresia and 12 (29.3%) had ileal atresia. Seventeen (41.4%) patients had TE. The median days to full feeds was 19; 28 in the TE group versus 16 in the non-TE group (P = 0.022). Four (9.7%) cases needed a re-exploration because of failure to start feeds; all in the non-TE group. The median LOS was 33 days for TE versus 22 days for non-TE (P = 0.101). Twenty-one cases (51.2%) developed a wound infection and showed a significantly longer median LOS of 29.5 versus 19 days (P = 0.019). Mortality was 7 (17.1%). Conclusions: TE did not show a superior outcome when compared to resection of the dilated bowel. It was associated with longer time to reach full enteral nutrition and longer LOS.

Malignant rhabdoid tumors (MRT) are uncommon, highly aggressive tumors arising usually from the central nervous system and kidneys. Nonrenal and noncentral nervous systems MRT are rare in neonates. To the best of our knowledge, only five cases of congenital MRT of neck have been described till date. We present a rare case of congenital MRT of the neck in a neonate along with review of literature.

Aim: The aim of this study was to study the awareness of medical research (MR) among resident doctors in a tertiary care hospital. Materials and Methods: This is a descriptive and cross-sectional study conducted for 30 days among the residents of paraclinical, surgical, and nonsurgical specialties, based on a customized self-prepared questionnaire. Convenience sampling was done. Undergraduates, interns, and faculty members were excluded from the study. Scoring was given from 1 to 10 based on their responses to the ten knowledge-based questions in the questionnaire. Data regarding the publication of research articles if any, obstacles in conducting research, and suggestions to improve the research awareness were recorded. Descriptive analysis of the data was done. Based on the scoring, they were divided into three groups: below average if the score is <5, average if the score is 5–7, and above average if the score is 8–10. Results: A total of 364 resident doctors were included in the study. They were divided into three groups which include paraclinical (n = 56, 15%), surgical (n = 132, 36%), and nonsurgical branches (n = 176, 48%). Scores for the three levels, i.e., below average, average, and above average were 0, 39% (n = 140), and 61% (n = 224), respectively. Forty-eight percent of participants had a publication. Obstacles for conducting the research included lack of time (55%), lack of interest (29%), lack of guidance (35%), and lack of material and teaching (45%). The majority (n = 300, 82%) suggested that conducting more interactive sessions or teaching programs may help in improving the awareness on research. Conclusions: Most of the resident doctors had a fair knowledge of MR but its application into practice was limited. Including research as a part of the medical curriculum, conducting educational programs or conferences oriented on research may improve the awareness about research.

Aim: The aim of this study was to evaluate the early indicators of sepsis (sepsis screening) and their statistical correlation with sepsis in neonatal abdominal surgery. Materials and Methods: A prospective observational study was performed on thirty consecutive neonate cases aged between 0 and 28 days with surgical abdomen at the Paediatric Surgery Department, ABVIMS and Dr. Ram Manohar Lohia Hospital, New Delhi. The study duration was 18 months. Septic screening was done in all neonates on day 0, 1, 3, 7, and 14 days of surgery with serum procalcitonin, C-reactive protein, total leukocyte count, immature/total neutrophil ratio, and microerythrocyte sedimentation rate. A septic screening-positive patient (three or more positive parameters out of five) was correlated with sepsis and analysis was done. Results: A total of 30 neonates of abdominal surgical cases were included consequently, out of which 56.7% (n = 17) were male and 43.3% (n = 13) were female. Maximum cases were of congenital diaphragmatic hernia 20% (n = 6) and then anorectal malformation 16.7% (n = 5). About 70% of neonates were sepsis screening positive. Fifty percentage of neonates were diagnosed to have sepsis on the clinical or laboratory findings, so sensitivity and specificity of sepsis screening were 93.33% and 40%, respectively. There was total 30% mortality in this study. Conclusion: Sepsis screening is an early marker of sepsis, which can be used to help in early detection of neonatal surgical sepsis and timely intervention that can lead to decrease mortality and morbidity in neonatal surgery.

Background: Two stage urethroplasty for proximal penile hypospadias is time consuming, expensive and; traumatic both for parents and phallus. On the other hand, single stage procedure technically demanding. We would like to describe Extended Ulaanbaatar Procedure (EUP) which is not a two stage procedure. Rather, might be called as 'extended single stage' procedure. In EUP we have done orthoplasty along with urethroplasty with preputial skin graft at same sitting as primary procedure keeping urination diverted through proximal hypospadiac meatus as “controlled fistula” which was closed after six months as secondary procedure. Methods: We operated on 35 patients of proximal penile hypospadias with moderate to severe chordee. Chordee was excised till correction of curvature. Two distracted cut ends of native plate was bridged with preputial skin graft (PSG) in between. Following that, silastic tube was placed over glandular plate as scaffold, on both cut ends of native plate and PSG. All the urethral plates and PSG were buried with tunica vaginalis flap before glanuloplasty. After six months, proximal “controlled fistula” was closed with scrotal dartos fascia and skin to join distal to proximal urethra. Results: Vertical meatus in glans was found in 32 patients. One patient had glans dehiscence, two patients had medium sized fistula, another two patients had stenosis in neourethra and six had suture track fistula. Twenty-nine patients had satisfactory curve with good flow in uroflowmetry as per nomogram at sixth month of follow up. Conclusion: In classic Ulaanbaatar procedure authors do distal urethroplasty and glanuloplsaty in 1^st stage following orthoplasty to avoid repeat trauma in glans in repeat procedures. Left over urethroplasty in classic Ulaanbaatar was done in 2^nd stage. However, in EUP; we did urethroplasty for full length following orthoplasty as primary procedure. This procedure is less invasive than two staged as we avoided repeat degloving and repeat dissection on operated tissues. Urethroplasty done as primary procedure shunned the need of repeat degloving, decreased the period of morbidity, stay, and cost of surgery. We also avoided problems of urination through not matured, long, neo-urethra. Similarly complications i.e disruption, stenosis in neo-urethra can be managed utilizing the advantages of urinary diversion.

Background: The number of times a research work gets cited by another article is one of the article-level metrics for assessing the quality of a research publication. Citation analysis by bibliometric review has been performed in several disciplines. The current study was aimed to systematically review the literature available on pediatric inguinal hernia since 1960 in terms of the 25 most cited articles in this field and analyze the bibliometric variables author and organizational collaborative patterns. Methods: Thomson Reuters Web of Science citation indexing database and research platform were used to retrieve the most cited articles in pediatric inguinal hernia (PIH) using appropriate search strings. The characteristics (name of authors, the total number of authors, the title of publication, journal of publication, year of publication, etc.) of the 25 top-cited articles were recorded. Specific bibliographical parameters were derived and analyzed. Visualization maps were generated using VOSviewer software. Results: The analysis revealed that the Journal of Pediatric Surgery was leading the choice of journal for publication. While most of the publications originated from the United States of America, Schier was the most influential author. Five of the eight top-productive authors are also the most connected. Conclusion: Articles on laparoscopic repair in PIH have been heavily cited. Following the United States of America, Turkey stands out as the topmost productive country in PIH. The publications on PIH show that “collaboration” is the bridging force between productivity and influence on the academic community.

Introduction: Single-nucleotide polymorphism (SNP) is a single-nucleotide change in a deoxyribose nucleic acid (DNA) sequence that occurs in >1% of population. Methylene tetra hydro folate reductase (MTHFR) C677T (rs1801133) and methionine synthase enzyme (MTR) A2756G (rs1805087) are two such SNPs occurring in coding sequence of the respective genes, which are frequently seen with neural tube defects (NTDs). MTHFR and MTR genes are involved in folate metabolism. The folate level in the course of pregnancy is treated as vital in the etiopathogenesis of NTDs. This study aims to explore the association of SNPs of both genes and red blood cell (RBC) folate levels in the predisposition to NTDs. Aims and Objective: The purpose of this investigation was to determine the relationship of NTDs with polymorphisms in MTHFR and MTR genotype and to estimate and compare the RBC folate levels in NTD patients and controls. Materials and Methods: A total of 397 individuals were enrolled (163 patients and 234 controls) for this observational study. Genotyping to find out MTHFR C677T and MTR A2756G was performed by polymerase chain reaction–restriction fragment length polymorphism technique from DNA extracted from the subject's blood. RBC folate level was estimated by chemiluminescence immunoassay method with the same blood sample. Results: The total RBC folate levels were significantly less among cases compared to controls (P = 0.020). A significant difference for RBC folate was observed between case and control groups of various genotypes of MTHFR C677T, except heterozygote CT (P = 0.459). Among MTR A2756G, genotypes with only homozygous AA have significant difference (P = 0.003) for RBC folate levels. Among different types of NTDs, there were no significant differences for RBC folate levels. Among MTHFR C677T, T allele possessed 1.9 times risk compared to C allele for the occurrence of NTDs. In MTR A2756G polymorphism, the odds of developing NTDs were 1.6 times in heterozygous AG compared to homozygous AA. Similarly, the risk for NTDs was three times higher in subjects with both heterozygous AG and CT genotypes compared to wild-type homozygous AA and CC genotypes. Conclusion: The total RBC folate levels were significantly less among cases compared to controls, and the genotypes had no such effect in decrease in RBC folate levels. The presence of mutant allele in homozygous or heterozygous condition for both SNPs had increased risk associated with NTDs.

Aim: Posterior cloacal malformations (PCMs) are distinguished from classical cloacal malformations by the posterior location of the common opening in the perineum. We aim to describe our experience of management of these rare and complex malformations. Methods: This study was a retrospective chart review of all patients with PCM who underwent treatment at Kalawati Saran Children's Hospital (KSCH), New Delhi, and Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, between 2013 and 2021. Individual anatomical variations and their impact on the clinical presentation, management, and final outcome (cosmesis and function) were recorded and analyzed. Results: During the study period, four girls with a median age of 2 (range: 0–5) years presented to us with PCM. Three patients were referred to KSCH as ambiguous genitalia whereas one patient was born at SGPGIMS with an antenatal diagnosis of “urorectal septal defect.” On examination, three patients had the urogenital sinus (UGS) opening immediately anterior to the normally located anal opening (2 orifices), whereas one had a single orifice at the normal location of the anus with the UGS opening in the anterior rectal wall. Associated anomalies included: (a) hydrocolpos (n = 3), which was managed by a tube vaginostomy; (b) urethral duplication with dorsal atretic urethra (n = 3); (c) uterine didelphys (n = 1); (d) bilateral grade 5 vesicoureteric reflux (n = 1); and (e) vaginal calculus (n = 1). Total urogenital mobilization (anterior sagittal approach) with feminizing genitoplasty was performed for patients with UGS and normal anus (n = 3). In the patient with a single opening at the normal location of the anus, posterior sagittal ano-recto-urethro-vaginoplasty was performed. At a median follow-up of 24 months, three patients are continent for urine and stool, whereas one patient is yet to be toilet trained. Conclusions: PCMs are unusual complex malformations that necessitate meticulous clinical examination, detailed diagnostic workup, and multistaged surgical management. Management should be tailored as per each individual patient's anatomy and clinical presentation for an optimal outcome.

Aim: We aimed to evaluate the parents' view of the cosmetic outcome after hypospadias surgery. Materials and Methods: Between January 2017 and December 2020, 70 patients aged between 2 and 7 years underwent surgical correction of hypospadias. Group 1 had 35 patients (coronal: 6, distal: 19, and mid-penile: 10) assigned for tubularized incised plate urethroplasty (TIPU) with modified foreskin reconstruction. Group 2 had 35 patients (coronal: 4, distal: 22, and mid-penile: 9) assigned for TIPU and circumcision. Parents were assessed with the Pediatric Penile Perception Score (PPPS). Results: Parent satisfaction for the position and shape of the urethral opening, shape of the glans, penile skin, and length exhibited no statistically relevant difference between the group. Parent satisfaction with the appearance of the genitals in Group 1 was relatively high 2.60 ± 0.55, as compared to Group 2 1.77 ± 0.49 which is statistically significant (P < 0.00028). Conclusion: Our experience shows good early cosmetic results of foreskin reconstruction and high parental satisfaction. Foreskin reconstruction should constitute a key element of the final result to restore a penis with an appearance as normal as possible.

Background: Neonatal sepsis term is an infection of newborns <28 days of age. It is a common cause of death in developing countries. The receptor-gamma receptor FCGR2A has been shown to be associated with neonatal sepsis. It is an activating receptor found in many cell types such as monocytes, neutrophils, macrophages, platelets, and others. The receptor has a polymorphism (single-nucleotide polymorphism rs1801274) in its gene (FCGR2A) that encodes either a histidine (H) or arginine (R) at amino acid position 131. There are many studies showing the impact of these FCGR2A polymorphisms on sepsis. Our study aims to determine the prevalence of Fc-gamma receptor FCGR2A (rs1801274) polymorphism in neonatal sepsis and control in Eastern UP populations. Patients and Methods: We conducted a cross-sectional descriptive study of 590 patients (310 healthy individuals and 280 sepsis patients) to determine polymorphisms in the CD32A coding region in neonates. All individuals were genotyped for a variant at position 131 of the FcγRIIA gene. Discussion: In our study, the prevalence of FcγRIIa polymorphism is more in neonates with sepsis than in noninfected neonates. It was observed that the heterozygous allele (AG) were significantly increased in septic neonates when compared to the normal. Conclusion: Our data indicate that FcγRIIA genotyping can be used as a marker of genetic susceptibility to sepsis.

Background and Aims: Dysplastic nubbin also referred to as testicular regression syndrome (TRS) is found in 5% of cases of the Non palpable testis (NPT). There is no consensus on the excision of the above and fixation of the contralateral solitary testis. We aimed to survey the prevalent practice of the same among members of the Indian Association of Pediatric Surgeons (IAPS). Methods: A structured questionnaire was sent through group e-mail and social media platforms to IAPS members to identify their practices in management. Results: A total of 132 surgeons responded to the questionnaire. Excision of intra-abdominal and inguinoscrotal TRS remnants was practiced by 84% (95% confidence interval [CI] 77%–89%) and 82% (95% CI 74%–87%). Fixation of contralateral solitary testis was practiced by 62% (95% CI 53%–70%) in the above scenario. Among the respondents, 30% reported encountering torsion of solitary testis during their career and this experience was a significant factor (P = 0.01) in deciding contralateral orchidopexy. Scrotal infection/necrosis was not encountered by a majority (72%) and it was not a deterrent factor in preventing contralateral orchidopexy (P = 0.68). Conclusions: The majority of pediatric surgeons favored the removal of intra-abdominal/inguinoscrotal TRS remnants identified during laparoscopy for NPT. A majority favored sutureless fixation of the contralateral solitary testis.

Background: Following esophageal atresia/tracheoesophageal fistula (EA/TEF) repair, the standard leak rate reported in the literature is 5%–10%, and stricture rate is 40%–72%. There is a global quest for surgical innovations to drive down these complication rates which can cause considerable morbidity. Methods: A prospectively maintained database of the senior author's patients who had esophageal atresia repair from 1995 to 2016 was reviewed. Two distinct innovations were implemented: (1) adequate or generous mobilization of the lower esophageal pouch and (2) a 2–5 mm slit in distal esophagus to widen its circumference. Results: Forty-three patients with EA/TEF were reviewed. Of those, 40 underwent primary repair. The median follow-up was 12.5 years (range 4–26 years). There were no anastomotic leaks and only 8 (20%) patients developed anastomotic strictures requiring dilations (1–5 dilations/patients). One patient (2.5%) had a recurrent fistula. One early mortality was recorded. At the latest follow-up, 35 (87.5%) patients had normal oral feeding, while 1 (2.5%) patient had occasional food sticking episodes. Four syndromic patients (10%) were on jejunal or gastrostomy feeding. Conclusion: An adequate or generous mobilization of the distal esophageal pouch, together with a 2–5 mm slit in the distal esophagus, achieves a tension-free and wide anastomosis. All anastomoses eventually narrow, sometimes just a little, and starting on a higher scale with a small slit, helps. These seemingly minor innovations, when used together, contributed to a substantially lower complication rate sustained over a 22-year period – no leaks and only 20% stricture rate.

Background: Double-J (DJ) stents were commonly used for internal drainage after major reconstructive procedures or in cases of obstruction and ureteral injuries. They should be removed or changed within the stipulated time; otherwise, they can lead to various complications such as stent occlusion, migration, breakage, encrustation, stone formation at either end of the stent, and entanglement of the two stents if bilateral stenting was done. The present study focuses on the complications and the management due to delay in the removal of the DJ stents due to the coronavirus disease-2019 pandemic. Materials and Methods: This is a cross-sectional study over a period of 9 months. Children <12 years were included in the study. The patients' demographic data, indication for DJ stenting, time gap between DJ stenting and removal, complication with delay in DJ stent removal, and its management were recorded. Indwelling duration for >4 months was considered a delay in removal. All patients were followed up for 3 months. Results: A total of 10 patients were included in the study. Encrustation, proximal migration, distal migration, knotting of the stent, and entanglement of the bilateral stents in the bladder were observed. These complications were managed by various endourological procedures such as ureteroscopy, percutaneous nephroscopic, and cystoscopic removal. During follow-up, all patients were symptom-free. Conclusion: Prolonged indwelling stents can cause various complications. Endourological procedures are an essential armamentarium for a pediatric surgeon to manage these complications. Proper patient counseling regarding indwelling stents and maintaining stent registry and sending automatic messages and e-mails to patients may prevent these complications.

Background: Congenital anomalies of the kidney and urinary tract (CAKUT) are a common cause of end-stage renal disease in children. While certain nephrogenic genes have been incriminated in these malformations, data to identify the frequency of gene polymorphisms in Asian Indian children with CAKUT are scarce. This study was done to identify the effect of polymorphisms in paired-box gene 2 (PAX2), bone morphogenetic protein (BMP)-4, angiotensin-converting enzyme (ACE), and angiotensin II receptor Type 2 (AGTR2) nephrogenic genes on the development of CAKUT. Materials and Methods: In this prospective cohort study, 158 children <12 years old (86 cases with CAKUT and 72 age-matched controls) were analyzed. DNA from both sets was extracted from peripheral blood using the Keygen DNA extraction kit, and single-nucleotide gene polymorphisms (SNPs) in PAX2, BMP-4, ACE, and AGTR2 nephrogenic genes were detected by polymerase chain reaction (PCR) using previously published primers and PCR conditions. Results: The presence of A allele SNP for AGTR2 gene at rs3736556 was found to be significantly correlated with the development of ureteropelvic junction obstruction and vesicoureteral reflux (VUR) with the TT allelic genotype having a lower incidence of pelviureteric junction obstruction (odds ratio [OR] 0.18 [95% confidence interval [CI], 0.06–0.55], P = 0.01) and VUR (OR 0.31 [95% CI, 0.11–0.91], P = 0.03). Furthermore, on substratification of the patients with the presence of the A allele of AGTR2, 24 out of 27 patients with scarring were found to harbor the D allele of the ACE gene, thus predisposing them to further renal damage. Conclusion: This study points to early evidence in the implication of nephrogenic genes in development as well as predisposition to renal injury in Asian Indian patients with CAKUT.

Pancreatoblastoma, an orphan disease, is the most common malignant epithelial neoplasm of the pancreas in children. With vague clinical features, diagnosis is made by radiological suggestions and histopathology. The presence of metastatic disease and inoperable/incomplete excision remains the poor prognostic markers. We present a rare instance of an adolescent who has survived metastatic pancreatoblastoma after neoadjuvant chemoreduction/complete surgical excision.

Clitoromegaly in female child is a rare condition. This is often associated with androgen excess in disorders of sexual differentiation usually congenital adrenal hyperplasia. Nonhormonal causes of clitoromegaly are rare and are commonly due to neurofibromatosis, epidermoid cyst, rhabdomyosarcoma, and very rarely hemangioma. Hemangioma is one of the common benign neoplasms of infancy, usually located in head, trunk, and extremities. We are reporting 4-month-old child with clitoromegaly with normal hormonal assay. Clitoromegaly was due to hemangioma. Histopathology of mass confirmed the diagnosis. Only five cases have been reported in literature till date.

Cervical thymic cysts are rare cysts which are seen in the first decade of life in males on the left side. The following is a case of a young female with a painless swelling on the right side of the neck. She was evaluated and underwent exploration of the neck and excision of the swelling.

Infantile myofibromas (IMs) are benign soft-tissue tumors of children. They are of fibroblastic–myofibroblastic origin and show considerable morphological overlap with other spindle cell neoplasms. Here, we present two cases of solitary myofibromas, one in a neonate and one in a 2-year-old girl. A 2-day-old girl presented with severe respiratory distress and died during intubation. At autopsy, a myofibroma involving the oropharynx with extension up to the larynx was noted. Second case was a 2-year-girl with a myofibroma in the hard palate. IM must be differentiated from other benign and malignant spindle cell tumors of infancy and childhood. Oropharyngeal myofibroma should be considered in the differentials of neonatal respiratory distress.

Acute graft-versus-host disease of the gastrointestinal tract (GI-aGVHD) is a rare condition, often requiring multiple laparotomies, ultimately leading to a burst abdomen. We report the successful use of a dermal regeneration template (DRT), combined with negative pressure wound therapy (NPWT) and skin grafts, to reconstruct the abdominal skin in an 11-year-old boy. The patient was a case of aplastic anemia, who underwent bone marrow transfers, the first of which failed and the second one was successful. He eventually developed gastrointestinal GVHD. Repeated laparotomies were done for recurrent intestinal obstruction. He also underwent resection anastomosis and end ileostomy, after which he developed an anterior abdominal wall defect due to a burst abdomen. After 12 months of management with multiple dressings, NPWT, and DRT, a stable coverage was achieved, without skin retraction. We report our experience in anterior abdominal wall reconstruction in a case of GI-aGVHD using DRT

Pediatric adrenocortical tumors (ACTs) are rare entities with an incidence of 0.2% of all pediatric tumors. Only two cases of antenatally detected ACT have been reported in the literature. Our case is the first report of an antenatally detected suprarenal mass which manifested with postnatal virilizing features and was proven to be adrenocortical adenoma on histology.

Colonic lipomas, even though rare, are the most common intramural tumor and the second-most common benign tumor of the colon after adenomatous polyps. We present the case of a 4-year-old boy with a large rectal lipoma causing anal prolapse, bleeding, and constipation, to discuss differential diagnostic problems and the proper management. A 4-year-old boy presented with symptoms of anal prolapse and constipation. Anal prolapse was accompanied by a tumor that occasionally was bleeding. Computed tomography was performed to determine the origin of the tumor and its relations with the surrounding tissues. Excision of the mass was performed through retraction through the anus and the anal wall was closed with sutures. Histopathological findings revealed a submucosal lipoma of a 5 cm diameter, with the erosions of the overlying mucosa. The patient was discharged after 3 days with no postoperative complications. Colonic lipomas, even though rare, are the second-most common benign tumor of the colon. Essential reasons for the resection are the potential complications, such as abdominal pain, change in bowel pattern, bleeding, obstruction, intussusception, perforation, and rarely transformation into a liposarcoma. Resection is performed endoscopically if the tumor is <2 cm in diameter and has a narrow base that allows safe ligation. Otherwise, the open procedure should be considered. In our case, prolapse of the mass through the anal canal allowed the transanal resection.

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

Primary renal primitive neuroectodermal tumors (PNET) are an extremely rare entity. The tumor is very aggressive presenting with metastasis and carries a dismal prognosis. We describe the case of renal PNET in an 11-year-old boy with a solid cystic lesion in the right kidney with a thrombus in the inferior vena cava and lung nodules, mimicking Wilms' tumor.

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.

Acute appendicitis is the most common surgical emergency in children. However, it is uncommon in neonates and infants. Often it can be challenging to diagnose acute appendicitis in children due to atypical clinical presentation and nonspecific symptoms. This is particularly true in neonates and infants. A high level of clinical suspicion is needed to diagnose infantile appendicitis. Delayed diagnosis is associated with higher perforation rates and increased disease-related morbidity. Imaging plays a key role in the prompt diagnosis of acute appendicitis and its complications. We report two cases of perforated appendicitis in babies <6 months old.