Choledochal cysts (CCs) are abnormal dilatations of the biliary system. Usually, CCs are classified into five types. The sixth type (Type VI) is an emerging and rare type, reported the first case in 1991. We report this rare CC, Type VI seen in our experience for the first time. We have also reviewed the literature; only 26 cases of Type VI were found, including adults and children, ever since the first case has been reported in 1991. To the best of our knowledge, this is the 11^th pediatric case report of a Type VI choledochal cyst.

Purpose: Analyze the surgical management strategy and the current effects on Quality of Life of diagnosed Esophageal Achalasia (EA) patients in our department, with an assessment of the incidence, clinical course, and related disorders. Methods: Data was based on a retrospective review of all children who received achalasia treatment at our department between 2008 and 2022. Using the Pediatric Quality of Life Inventory Generic Core (PedsQL) Scales and the Pediatric-QL Gastrointestinal Symptoms Module (GI-PedsQL), we evaluated the long-term effects of this diagnosis on our patients' quality of life. Results: Esophageal achalasia (EA) was identified in a total of 13 cases (F: 7; M: 6). The mean patient delay was 27.90 months, and the median age was 6.5 years at diagnosis (2 months to 15 years). Regurgitation was the most frequently observed symptom, and the median follow-up was 7.32 (3- 30.7) months. There were descriptions of associations with Allgrove syndrome (n=7) and Down syndrome (n=1). All patients were treated by Open Heller myotomy (OHM) with one patient (n=1) initially treated by pneumatic dilatation (PD). In all 13 laparotomy myotomies, simultaneous fundoplication was performed using the DOR (n=12) and THAL (n=1) techniques. At follow-up, 12 patients had complete symptom relief with 1 patient exhibiting a persistence of symptoms. All 13 patients were prospectively contacted with a median time from latest check-up to date of inclusion of 2.9 (0.3-8.8) years. In comparison to data from a control group of EA patients, the overall PedsQL score of our patients was 72/100 (±17), with higher established scores in physical and emotional functioning but lower scores in social and school settings. On the other hand, when compared to a control group presenting with a gastroesophageal reflux disease (GERD), the GI-PedsQL overall score in our patients was higher with a 76/100 (±14.9), but they scored significantly lower in the dimensions: Foods and drinks limitations, difficulty swallowing, heartburn and vomiting. Discussion: Our study group's characteristics join most of the published literature with 54% of our patients presenting with Allgrove syndrome but it also joins the much rarer descriptions of Achalasia and Down's syndrome association with a singular case. The clinical course of our diagnosis process mainly consisted of timed esophagram and UGI endoscopy, lacking significantly in HREM evaluations but was efficient enough in guiding the surgical management. Our findings confirm the safety and effectiveness of Open Heller's myotomy with a success rate of 92.3% However, we concede that a laparoscopic approach should be further adopted if no contradiction is found. Moreover, we presented insight on long term QoL of patients posteriori to HM which was in concordance with other global experiences where patients reported dissatisfaction with their health and limited lifestyle. This significant negative impact was prominently present in comparison to healthy control groups as well as patients with inflammatory bowel diseases and compels a much needed discussion on the major chronic repercussions of Esophageal Achalasia in children. Conclusion: Even when managed by interdisciplinary teams of professionals, managing esophageal achalasia in specialized institutions is still a subject of debate. Early pediatric diagnosis is best achieved with routine physiological function tests and HREM. Moreover, more research is required to assess long-term Quality of Life. Standardized lifelong follow-up and qualified resources, both human and technological, are required to improve postoperative outcomes.

Aims: The aim of this study was to compare the immediate and long-term outcomes after high spermatic vessel ligation (HSVL) and low spermatic vessel ligation (LSVL) in a high undescended testis (UDT) model in rats. Materials and Methods: A prospective randomized controlled study was conducted on 24 male Wistar rats. The rats were randomly divided into three groups. Group A underwent a sham laparotomy and acted as the control. Group B underwent HSVL of both testicular vessels. Group C underwent LSVL of both testicular vessels. Each group was again subdivided into two subgroups. One sub-group underwent blood collection and testicular biopsy of both testes 24 h after the procedure to demonstrate immediate changes. Other subgroups underwent blood sample collection and testicular biopsy of both testes on day 50 following the procedure for hormonal changes and long-term changes. Results: All the testes in HSVL showed atrophy (100%) in the long term, whereas LSVL showed atrophy in 12.5% of testes, even though both groups showed adequate neovascularization. Testes in HSVL showed poor bleeding on incision at both 24 h and day 50. On histology, 75% of testes in HSVL showed complete necrosis, and 50% in LSVL showed partial necrosis at 24 h. On day 50, all the testes in HSVL (100%) showed complete necrosis with dystrophic calcification, whereas all the testes in LSVL showed normal histology with good maturation of seminiferous tubules. There was no significant difference in testosterone levels between both groups. Conclusions: Both immediate and long-term changes following LSVL showed an increase in blood flow to the testis after ligation through collaterals and reverses early ischemic changes to the testis. Given the higher testicular atrophic rate after HSVL, LSVL or at least low ligation can be preferred for the management of high intra-abdominal UDT.

Aim: In addition to the well-known toxicities of treatment, survivors of pediatric solid tumors can also develop other health-related conditions. They may either be an indirect consequence of therapy or could be unrelated to their prior history of malignancy. We aim to evaluate the nontoxicity related health conditions in survivors of pediatric solid tumors. Materials and Methods: The study included a cohort of hepatoblastoma (HB), Wilm's tumor (WT), and malignant germ cell tumors (MGCT) survivors registered at pediatric surgical-oncology clinic from 1994 to 2016. Follow-up was done according to standard protocols and children were evaluated at each visit for any health-related conditions. Results: Of the survivors, 318 survivors, comprising of 48, 81, and 189 survivors of HB, MGCT, and WT, respectively, were included in the analysis. We found 20.8% of patients with HB, 11.1% of patients with MGCT, and 16.4% of patients with WT to report nontoxicity-related health issues. A high prevalence of surgical conditions (3.4%), secondary malignancies (1.2%), gynecological conditions in girls (16.9%), tuberculosis (1.2%), gallstone disease (0.9%), pelvi-ureteral junction obstruction (0.9%), and neurological issues (0.9%) was noted. Two presumed survivors had died, one due to a late recurrence and the other due to a secondary malignancy. Conclusions: A high prevalence of medically or surgically manageable conditions makes it imperative to keep these children under follow-up to address any health-related conditions they may subsequently develop.

Background: Preoperative anxiety (PA) in children is a common phenomenon associated with various negative patient outcomes. Allaying PA is accepted as a standard of care, but its use is not universal and often overlooked. This survey is designed to evaluate the nationwide current practice patterns and attitudes of anesthesiologists toward the practice of allaying PA in children. Materials and Methods: A questionnaire of 25 questions, including information on methods of relieving PA in children, reasons for noncompliance, and associated complications, was framed. It was circulated among members of the Indian Society of Anaesthesiologists through an online survey of Google Forms and manually. Results: Four hundred and fifty anesthesiologists were surveyed. Responses were predominantly from anesthesiologists practicing in medical colleges across the country. Although 97% of the surveyed respondents practiced anxiety-relieving strategies, only 37% used it consistently. Seventy-three percent of anesthesiologists practiced both pharmacological and nonpharmacological techniques. The most common reason for avoiding premedication was an anticipated difficult airway (88%). Inadequate sedation was a commonly reported problem. Ninety-five percent of participants felt that PA-relieving strategies should be integral to pediatric anesthesia practice. The most common reason for not following these practices was an inadequate hospital infrastructure (67%). Ninety-seven percent of the participants believed that more awareness is required on this crucial perioperative issue. Conclusion: Only 37% of the surveyed anesthesiologists consistently used some form of PA-relieving strategy and the practice varied widely. Further improvement and team approach involving anesthesiologists, surgeons, and nurses is required to ensure the quality of pediatric PA-relieving services and establish it as a standard of care.

Aim: Patients with hypospadias often present with voiding difficulties after successful repairs. We sought to analyze the degree of uroflowmetric anomalies that may be present in children with hypospadias before a surgical repair. We, thus, undertook this study to compare the pattern of preoperative uroflowmetry study in hypospadias patients and a comparable age-matched control group. Materials and Methods: A total of 90 children underwent preoperative uroflowmetry in the Department of Pediatric Surgery at All India Institute of Medical Sciences (AIIMS), Jodhpur, India, between January 2019 and December 2020. Thirty patients with hypospadias and sixty age-matched controls who presented to the outpatient department without any associated urological or neurological anomalies were included in the study. Uroflowmetry parameters such as maximum urine flow rate (Q_max), average urine flow rate (Q_avg), voided volume (VV), urination duration, duration of reaching maximum speed, and urine flow curves of the cases and the control group were measured and compared. Results: The median age of patients at the time of uroflowmetry in the hypospadias group was 7 years, while the same for the control group was 7.5 years. Median maximum urinary flow rates (Q_max) (10.7 vs. 14.45 mL/s, P = 0.01278), average urinary flow rates (Q_avg) (6.5 vs. 8.5 mL/s, P = 0.0124), the ratio of maximum urinary flow rates with VV (Q_max/VV) (0.043 vs. 0.053, P = 0.0264) was found to be significant (P < 0.05). These values were significantly lower in the hypospadias group (P < 0.05). The voiding time (43.5 vs. 30 s, P = 0.0285) was significantly higher in the hypospadias group. However, there was no difference in the VV per micturition (219.5 vs. 270.0 mL, P = 0.40) and time to maximum flow rate (10 vs. 10, P = 0.43). Flow curve pattern analysis revealed plateau-shaped curves in 60% of the hypospadias group compared to 27% in the control group, while bell-shaped curve was seen in 37% of the hypospadias group as compared to 65% in the control group which were statistically significant (P = 0.003415). No statistically significant association was found between meatal localization and the uroflowmetry parameters. Conclusion: Children with hypospadias have abnormal uroflowmetry even before surgical correction and have a significantly low maximum urine flow rate. These uroflow anomalies may be due to meatal stenosis or hypoplastic proximal urethra. We postulate that these preoperative abnormal uroflow patterns in patients with hypospadias may contribute to voiding difficulties in repaired hypospadias cases. A greater understanding of the factors behind these uroflowmetric anomalies may allow surgeons to proactively tackle these intraoperatively, leading to better outcomes for patients with hypospadias.

Introduction: Malrotation is a congenital anatomical anomaly that affects the normal positioning of the intestines. Traditional management of malrotation, as described by Ladd, consists of detorsion of the volvulus if present, division of Ladd's bands, widening of the mesenteric root, proper positioning of the small and large bowels, and a prophylactic appendectomy. This study was done to determine whether appendectomy should be an integral part of the Ladds procedure or if it can be avoided. Materials and Methods: This retrospective observational study was conducted in one pediatric surgical unit in the tertiary care center of North India. All the cases of malrotation of the gut managed from January 2008 to December 2018 were reviewed. The details of the patients were recovered from the electronic data recording system of the hospital and manual operation theater records. The cases that have a follow-up of <5 years were not included in the study. The details were charted in an Excel Sheet for the analysis. No statistical test was performed because there was no event in patients in whom prophylactic appendectomy was not done. Results: The data analysis revealed that a total of 66 malrotation patients were managed during this period, those who fulfilled the inclusion criteria. Among 66 cases included in the study, in 41 cases, prophylactic appendectomy was done, whereas in the rest 25, prophylactic appendectomy was not done. During the follow-up, none of the patients in whom the appendix was preserved presented with signs or symptoms related to appendicitis or any other complications. Conclusion: Ladds procedure without appendectomy can be performed in view of the potential use of the appendix in the future, and with the fact that in the current era of advanced medicine, appendicitis can be diagnosed early due to the advancement of imaging and better record keeping.

Introduction: A scientometric analysis was conducted to characterize the global research publications in extrahepatic portal venous obstruction (EHPVO), and state-of-the-art visualization graphics were generated to provide insight into specific bibliometric variables. Materials and Methods: The Web of Science database was accessed for research productivity and bibliometric variables of countries, institutions, authors, journals, and content analysis of top-20 cited documents were performed. Collaborative networks and co-occurrence of keywords map were generated using VOSviewer software. Results: Two hundred and sixteen records were retrieved with an annual growth rate of 2.53%. India is the leading country in productivity (n = 4339), followed by the USA and China. Post Graduate Institute of Medical Education and Research, Chandigarh, was the top productive institute. Sarin SK was the most prolific author, having the highest citations received and h-index. The hotspot topics were “portal hypertension,” “cirrhosis,” “children,” “biliopathy/cholangiopathy,” “liver fibrosis,” and “liver transplantation” as per keyword co-occurrence networking. J Gastroenterol Hepatol had the most publications of EHPVO research as well the h-index. Regarding collaborative network mapping, the USA and Primignani M were the significant nodes among country and author, respectively. Conclusion: EHPVO research publication volume is low but is gradually progressing with dominant contributions from Indian institutes and authors. Most highly cited articles are of low level of evidence, and multi-institutional collaborative research can be the way forward.

Background: Most congenital pulmonary airway malformations (CPAMs) are detected antenatally. The majority of newborns are asymptomatic. Patients are prone to subsequent respiratory complications and to a lesser extent malignant transformation remains concerning. In Israel, until 2013, pediatric surgeries were performed by thoracotomy. To minimize its morbidity, we introduced thoracoscopy using a mentorship approach. We present our experience with thoracoscopic resections coordinated by the mentorship of a pediatric worldwide leader in his field and compare our results with resections performed by thoracotomy. Materials and Methods: A retrospective review of records of children operated between 2013 and 2020 was conducted. Data were compared using t-test for quantitative variables. Results: Fifty patients were operated by thoracoscopy with a median age of 4 years, a thoracoscopic lobectomy performed in 68%. There was no conversion with a median length of stay (LOS) of 3½ days. Thirty patients were operated by thoracotomy by a thoracic surgeon with a median age of 3.5 years. A lobectomy was performed in 87% with a median LOS of 7 days. Conclusions: Thoracoscopic lobectomy is a technically demanding procedure with a long learning curve, strongly related to the low volume of cases. The role of a mentorship program in acquiring those surgical skills is crucial through standardization of the technique applied and supervised by the mentor. Early thoracoscopy for congenital pulmonary lesions at an early age can be achieved with a low conversion rate and minimal complications creating a change in the paradigm of practice when considering surgery for CPAM in Israel.

Scrotal swellings in neonates are very common. A 25-day-old male child had right-sided firm, nontender scrotal swelling. Ultrasound scan (USS) diagnosed as meconium periorchitis (MPO). At 70 days of age, repeat USS and plain abdominoscrotal radiograph confirmed MPO, and surgery was avoided. Radiological diagnosis and management of MPO are discussed.

Idiopathic intussusception is the most common form of intussusception in infants and young children. In older children and adults, intussusception being rare, the lead point is usually an underlying bowel pathology (Meckel's diverticulum, hemangioma, carcinoids, polyps, submucous lipomas, etc.) and these are called pathological lead points (PLP's). The management of an obese child with recurrent abdominal pain for over 2 years, diagnosed with ileo-ileal intussusception due to submucosal lipoma is reported here.

Bardet–Biedl syndrome is an autosomal-recessive ciliopathic disorder affecting multiple organ systems. Characteristic features include progressive retinal dystrophy, obesity, polydactyly hypogonadism, mental retardation, and renal disorders. Other manifestations include congenital heart diseases, hepatic fibrosis, ataxia, and diabetes. Approximately 30% of patients with Biedl–Bardet syndrome (BBS) have hepatobiliary disorders such as periportal fibrosis, nonalcoholic fatty liver disease, and cystic dilation of the bile ducts. The association of BBS with choledochal cysts (CDC) is extremely rare. Here, we report a case of a 14-year-old boy with a novel variant of BBS and associated type IV CDC. The patient was managed surgically with CDC excision and Roux-en-Y hepaticojejunostomy.

Bronchoesophageal fistulas (BEFs) are rare malformations characterized by abnormal communication between the bronchus and the esophagus. Herein, we report a successful management of a child with BEF discovered as an intraoperative surprise and associated with the short gut, annular pancreas, and disorder of intestinal fixation.

Pulmonary actinomycosis is an extremely rare disease in children. The diagnosis is challenging as the clinical presentation and radiological investigations may be atypical. We report a case of a pulmonary lesion extending to the chest wall posing as a malignant lesion. Biopsy revealed colonies of Actinomyces. Antibiotic therapy resolved the mass. A rare diagnosis of pulmonary actinomycosis should be kept as a differential diagnosis of such a mass in children.

A 5-day-old male presented with bilious vomiting, a grossly distended abdomen, and passage of a small amount of stool. The anal opening was at a normal position. X-ray abdomen showed a large bowel loop with a single air-fluid level occupying more than half of the abdominal width. On laparotomy, the ascending colon was dilated to form a pouch-like structure, and the ileum and appendix were opening into it. Colon distal to pouch was present as microcolon. Histopathology of the dilated segment was suggestive of congenital segmental dilatation (CSD). In CSD, the distal bowel is of normal caliber. This is a rare case of CSD of ascending colon with distal microcolon.

Biliary cystadenoma, a rare potentially malignant hepatic cystic lesion, is characterized by multiloculations and septations. It is common in middle-aged females (about 5% of nonparasitic liver cysts); only 12 cases are described in children. We report a rare case of hepatic biliary cystadenoma in a 3-year-old girl, with a gradually increasing lump in the right upper abdomen. Complete excision with a healthy liver margin was done.

Diphallia (penile duplication) is a rare congenital malformation with an incidence of about 1 per 5–6 million newborns. The severity of diphallia varies from a small accessory penile-like tissue to complete true penile duplication with other deformities, usually involving the urogenital, gastrointestinal, and musculoskeletal systems. Pseudodiphallia, as a rare kind of diphallia, is characterized by a small accessory penile-like tissue without a normal penile anatomy structure. A 5.5-year-old male child was brought to the pediatric surgery outpatient department by the parents with complaint of difficulty in retracting the prepucial foreskin and the presence of some growth near the glans. There were no other complaints in specific. Clinical examination revealed foreskin retractable with difficulty and small conical lump smaller than the original glans approximately size ~1 cm diameter at the base attached horizontally at the left side of the original glans at the coronal sulcus and visible incomplete clefting in between the 2 glans visible from the aerial view. After approval from anesthetist, the patient was operated under general anesthesia by excision of pseudodiphallia. Urethral catheterization and circumcision of the penis after taking informed parental consent. Postoperatively, the period was uneventful. The patient responded well to the symptomatic treatment and was orally allowed on the same day evening. Urinary catheter was removed on 5^th postoperative day. The patient was discharged on oral symptomatic medication and was advice for follow-up.

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.