Comparison of the Surgical Outcomes in Neonates with Left-sided Congenital Diaphragmatic Hernia with Only Skin Closure versus Abdominal Muscle Closure.

Aim: This study aims to compare the outcome of neonatal left congenital diaphragmatic hernia (CDH, Bochdalek type) repair through laparotomy with and without abdominal muscle closure. Materials and Methods: This retrospective study was conducted between January 2012 and May 2021 at a neonatal surgical unit of a Tertiary Care Center. Demographic details, preoperative management, Two-dimensional-echo, intra-operative findings, postoperative course, and follow-up data were collected and analyzed. Results: The study group comprised 50 neonates with a mean standard deviation (SD) age at admission: 4.44 (5.12) days, male: female ratio of 3:2, and mean (SD) weight: 2.73 (0.51) kg. Following repair of the diaphragmatic defect through laparotomy, 26 (52%) underwent skin closure alone, whereas 24 (48%) underwent abdominal muscle closure. Postoperatively, there was a significant fall in the level of platelets (P = 0.021), increase in pressure support by at least 4-5 cm H2O (P = 0.027), and increase in the blood urea (P < 0.001), creatinine (P = 0.005), lactate (P = 0.019), and acidosis (P = 0.048) in the muscle closure group. Although not statistically significant, there was a fall in the urine output and blood pressure in this group. There was no significant difference in the duration of inotropes. Mortality was 8 (32%) in the skin closure group, and 14 (61%) in the muscle closure group (P = 0.05). Conclusions: Neonates undergoing left CDH repair through the abdominal route with skin closure alone, had better survival, as well as hematological, renal, and ventilatory parameters than those who underwent muscle closure. It is a useful surgical modification to improve outcome in centers with limited facilities.

Polymeric immunoglobulin receptor and galectin-3-binding protein are raised in biliary atresia: Reveals a proteomic-based study.

To identify and evaluate differentially expressed plasma proteins in biliary atresia (BA), we performed plasma proteome profiling using liquid chromatography with tandem mass spectrometry (LC-MS/MS) in 20 patients with BA and 10 control children. Serological assays validated the most significant and highly upregulated proteins in a cohort of 45 patients and 15 controls. Bioinformatics tools were used for functional classification and protein-protein interactions of differentially expressed proteins (DEPs). Of 405 proteins detected in patients and 360 in controls, 242 proteins, each with ≥2 unique peptides (total of 3230 peptides), were common in both groups. Compared to controls, 90 proteins in patients were differentially expressed and were dysregulated. Twenty-five were significantly upregulated with polymeric immunoglobulin receptor (PIgR), galectin-3-binding protein (Gal-3BP), complement C2, the most prominent, and 15 had low expression. The bioinformatic analysis revealed functional interaction between DEPs and their role in an inflammatory immune response. Enzyme immunoassay for PIgR and Gal-3BP in patients' plasma showed their levels raised significantly (p = 0.0021 and p = 0.0369, respectively). The PIgR and Gal-3BP are novel proteins upregulated in BA and may be tested further for their utility as potential circulating disease biomarker(s). SIGNIFICANCE: The study shows that plasma PIgR and GAL-3BP levels are significantly raised in infants with BA within the first 3 months of life. If tested in a larger cohort, these proteins may be found to have their diagnostic potential and utility as disease biomarkers. The study also provides valuable information on the involvement of several DEPs in innate immune response, chronic inflammation, and fibrosis. This strengthens the hypothesis that the immune-mediated inflammatory processes are responsible for the progressive nature of BA.

Association between intraoperative anatomical variation and histopathological parameters in cases of ureteropelvic junction obstruction in children: A cross-sectional study.

Introduction: The intraoperative anatomical findings (IOAF) of all ureteropelvic junction obstruction (UPJO) cases are not identical. Moreover, there is also controversy in the literature regarding histopathological (HP) findings in cases of UPJO. In the present study, we evaluated different IOAF and assessed their association with specific HP parameters. Materials and Methods: This was a cross-sectional study set-up, which was carried out in a tertiary care centre. Children with UPJO who underwent surgery between 2017 and 2020 were enrolled. The following IOAF were noted: Type of pelvis (extrarenal or intrarenal), insertion of the ureter (high or normal), presence of lower pole crossing vessel (CV), negotiation of UPJ segment with double J stent (3 Fr) and length of internal narrowing (LIN) at UPJ. The resected segment of UPJ was assessed at three levels (pelvis, UPJ and ureter) for various HP parameters including fibrosis, oedema, inflammation and smooth muscle hypertrophy (SMH). Results: Thirty-nine children were included in the study with a mean age of 31 months. The summary statistics of IOAF were intrarenal pelvis in 5 cases, high insertion of the ureter (HIU) in 9, CV in 6, negotiable UPJ in 23, and 16 cases showed LIN >1 cm. All cases showed SMH at the pelvis region and SMH with fibrosis at the UPJ region. At the pelvis region, there was an association between (1) HIU with oedema and chronic inflammation (CIF), (2) CV with CIF and (3) LIN with CIF and SMH. At the UPJ region, there was an association between (1) CV and negotiable UPJ with less fibrosis and (2) LIN with SMH. At the ureteric end, CV showed an association with less fibrosis and more CIF. Conclusion: All UPJO cases have some common HP findings. Although, some particular IOAF, i.e., presence of CV, negotiable UPJ, HIU and LIN showed association with specific HP parameters.

Debate 2: Is the Management of Childhood Empyema Primarily Medical, or Surgical?

The management of empyema thoracis has changed in the past 1-2 decades with the advent of video-assisted thoracoscopy (VATS), the surgeon's weapon and fibrinolytic agents, the physician's weapon. Inappropriate use of either and inability to accept their failure in some cases cause prolonged morbidity in an unsuspecting patient. VATS has been shown to be very effective in stage 2 empyema and is associated with early amelioration of symptoms and early discharge from hospital. Open thoracotomy still plays an important role in the management of delayed and complicated cases of empyema and has always produced good results. Some complications seen by surgeons are related to previous interventions and delayed referral. Lung status plays an important role in post operative recovery. In patients requiring intervention, both medical and surgical options should be considered without bias early in the management and discussed with care-givers to give best outcome.

Long-Term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged ⩾6 Years with Cystic Fibrosis and at Least One F508del Allele: A Phase 3, Open-Label Clinical Trial.

Rationale: A 24-week, phase 3, open-label study showed elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was safe and efficacious in children aged 6-11 years with cystic fibrosis (CF) and one or more F508del-CFTR alleles. Objectives: To assess long-term safety and efficacy of ELX/TEZ/IVA in children who completed the pivotal 24-week phase 3 trial. Methods: In this phase 3, two-part (part A and part B), open-label extension study, children aged ⩾6 years with CF heterozygous for F508del and a minimal function CFTR mutation (F/MF genotypes) or homozygous for F508del (F/F genotype) who completed the 24-week parent study received ELX/TEZ/IVA based on weight. Children weighing <30 kg received ELX 100 mg once daily/TEZ 50 mg once daily/IVA 75 mg every 12 hours, whereas children weighing ⩾30 kg received ELX 200 mg once daily/TEZ 100 mg once daily/IVA 150 mg every 12 hours (adult dose). The 96-week analysis of part A of this extension study is reported here. Measurements and Main Results: Sixty-four children (F/MF genotypes, n = 36; F/F genotype, n = 28) were enrolled and received one or more doses of ELX/TEZ/IVA. Mean (SD) period of exposure to ELX/TEZ/IVA was 93.9 (11.1) weeks. The primary endpoint was safety and tolerability. Adverse events and serious adverse events were consistent with common manifestations of CF disease. Overall, exposure-adjusted rates of adverse events and serious adverse events (407.74 and 4.72 events per 100 patient-years) were lower than in the parent study (987.04 and 8.68 events per 100 patient-years). One child (1.6%) had an adverse event of aggression that was moderate in severity and resolved after study drug discontinuation. From parent study baseline at Week 96 of this extension study, the mean percent predicted FEV1 increased (11.2 [95% confidence interval (CI), 8.3 to 14.2] percentage points), sweat chloride concentration decreased (-62.3 [95% CI, -65.9 to -58.8] mmol/L), Cystic Fibrosis Questionnaire-Revised respiratory domain score increased (13.3 [95% CI, 11.4 to 15.1] points), and lung clearance index 2.5 decreased (-2.00 [95% CI, -2.45 to -1.55] units). Increases in growth parameters were also observed. The estimated pulmonary exacerbation rate per 48 weeks was 0.04. The annualized rate of change in percent predicted FEV1 was 0.51 (95% CI, -0.73 to 1.75) percentage points per year. Conclusions: ELX/TEZ/IVA continued to be generally safe and well tolerated in children aged ⩾6 years through an additional 96 weeks of treatment. Improvements in lung function, respiratory symptoms, and CFTR function observed in the parent study were maintained. These results demonstrate the favorable long-term safety profile and durable clinical benefits of ELX/TEZ/IVA in this pediatric population. Clinical trial registered with www.clinicaltrials.gov (NCT04183790).

Unusual manifestations of Peutz-Jegher's syndrome in children.

PeutzJegher's syndrome (PJS) is a rare, autosomal dominant disease, characterized by gastrointestinal (GI) polyps and perioral hyperpigmentation along with the increased risk of certain malignancies. In children, the most common presentation is recurrent intussusception due to polyps. These polyps can involve any part of the GI tract and can present with a variety of clinical presentations. Usually, these polyps can be removed endoscopically but often require surgical excision also. In this report, we discuss two children of PJS with uncommon presentations, one presented with retrograde intussusception and another with gastric outlet obstruction. The first child underwent laparoscopy and another required open surgery with intraoperative enteroscopy.

Primary Repair of Pediatric Posttraumatic Complete Bladder Neck Horizontal Transection: Our Experience.

Complete transverse transection just below the bladder neck is extremely rare. We present two such cases with associated pelvic fracture following trauma. Both underwent early primary vesicourethral anastomosis with no postoperative complications and are continent in the follow-up.

Duodenal and Pyloric Web in Children: Clinical Presentation and Management.

Background: Duodenal and pyloric web (DW/PW) can present at any age, symptoms depend upon the location of the web along with the presence and size of the opening in the web. The surgical management is not straightforward always. Here, in this study, we aim to assess clinical characteristics, management, and outcome of children with DW/PW. Materials and Methodology: This was a retrospective study from 2005 to 2019, and data were collected from record registers. All children of DW/PW presented between this duration were included in this study. Results: A total of 45 patients (age range = 1 day to 11 years) included in the study, 40 had DW while 5 had PW. Seven patients were diagnosed antenatally and 20 patients had associated congenital anomalies. Most patients presented with vomiting either bilious or nonbilious. Plain X-ray was sufficient for the diagnosis in 60% of patients, the rest diagnosed on contrast study. The web excision and pyloroplasty were done for PW. The web excision and Heineke-Mikulicz type enteroplasty was the preferred surgery for DW but some patients were required Kimura's duodeno-duodenostomy. For postoperative nutrition, enteral feeding was established through the placement of a feeding tube beyond anastomosis. Ten patients died due to septicemia and associated anomalies. Four patients had a minor leak which was managed by conservative means. Four patients required redo surgery, adhesive obstruction was the most common indication. During follow-up, all 35 patients were doing well with no major complaints. Conclusion: DW/PW has different presentations as compared to other intestinal atresia and can present at any age. A contrast study confirms the diagnosis when plain X-ray is inconclusive. Associated anomalies and septicemia are the poor prognostic indicators. Postoperative enteral feeding helps in maintaining adequate nutrition and improves the outcome even in children with a minor anastomotic leak.

Reverse Gastric Tube Esophagoplasty with and without Lower Esophageal Stump Wrap - Comparison of Outcome.

Aim and Objectives: The aim of the study is to compare the outcome in children born with long-gap esophageal atresia following reverse gastric tube esophagoplasty (RGTE) with or without the lower esophageal stump as a "fundoplication" wrap. Materials and Methods: All children who underwent RGTE between 2008 and 2018 were retrospectively analyzed. Patients in whom the lower esophagus (LE) had been excised as is done routinely in RGTE (Group 1) were compared with those where the LE was wrapped partially or completely around the intraabdominal neo-esophagus (Group 2). Both vagal nerves were preserved to the extent possible. Complications and final outcome, including weight and height centiles were assessed. Follow-up upper gastrointestinal contrast study and reflux scans were studied. Results: Nineteen patients (mean age: 15.78 ± 5.02 months [range 10-30 months] at RGTE) were studied; nine in Group 1 and ten in Group 2. Both groups had similar early postoperative complications as well as the requirement of dilatation for anastomotic stricture. Dysphagia for solids was noticed in two patients with complete lower esophageal wrap (n = 4), one requiring removal. More patients in Group 2 had absent reflux (n = 7) compared to Group 1 (n = 3) (P = 0.118). At a mean follow-up period of 45.75 ± 18.77 months (14-84 months), Group 2 children reached better height and weight percentiles compared to Group 1. Conclusion: We have described a novel method of using the LE as a "fundoplication" wrap following RGTE. Vagi should be preserved. Those with complete esophageal wrap may develop dysphagia to solids and this is, therefore, not recommended. Lower esophageal wrap patients appeared to have a better outcome in terms of growth and less reflux.

Factors affecting stress levels in parents of surgical neonates: A prospective observational study.

BACKGROUND: There is a paucity of research focusing on the stress levels in parents of newborns undergoing surgery. Resource challenged systems have to deal with overcrowding, a shortage of workforce along with demographic and socioeconomic issues like delayed presentations and out of pocket expenses. The primary objective of this study was to understand the factors associated with stress in the parents of these congenitally malformed neonates. METHODOLOGY: This was a prospective cohort study, which was conducted in a neonatal surgical ICU of a tertiary care teaching hospital. Factors affecting stress levels in parents of surgical neonates were studied in 100 participants. A multi-dimensional questionnaire - The PSS: NICU score was utilized in the study. The parents were interviewed on Day 3-5 after surgery. RESULT: 59% of the respondents were fathers. The majority of the parents were in the age bracket of 24 to 35 years. The mean scores for the subscales sights and sounds, looks and behavior and alteration in the parental role were 3.24±0.8, 3.52±0.63, 3.55±0.8 and 2.8 ± 0.9 respectively. The highest level of stress was found in the domains of alteration of parental role and infant appearance and behavior. Comparisons showed significantly higher maternal scores in all the domains. Overall stress scores were highest for abdominal wall defects. CONCLUSION: Parents of neonates undergoing surgery suffer from significant stress levels and appropriate counseling targeted towards specific stressors is required to allay this important parental issue.

External and internal parasitic conjoined twinning: Diverse presentation and different surgical challenges.

Background: Parasitic conjoined twin is a rare but well-known entity with unclear embryopathogenesis. Abnormal conjoined twinning can result in an externally attached parasitic twin (PT), an enclosed foetus in foetu, or a mature teratoma. The treatment requires complete excision and reconstruction of local anatomy which is not always straightforward. Materials and Methods: PT cases presenting over 12 years were analysed. Patients with complete data, histopathological diagnosis and follow-up were included in the study. During follow-up, specific complications and related investigations were considered. Results: A total of five patients at four different sites were identified: two retroperitoneal foetus in foetu and three externally attached PTs which were located in the lumbar region, sacrococcygeal area and on the lower anterior abdominal wall. All patients underwent complete surgical excision. In foetus in foetu cases, the blood supply was directly from the aorta with a short stump while the three externally located ones required meticulous and careful dissection with the reconstruction of local anatomy. Conclusion: Parasitic conjoined twinning can present at different sites and surgical challenges vary accordingly. For surface lesions, reconstruction may be as complicated as excision. Prognosis can be affected by the excellence of anatomical restoration. Long-term follow-up is essential to address problems specific to the site of lesion and method of surgical reconstruction.

A higher tumor volume and undernutrition at diagnosis adversely affect the survival of children with Wilms tumor: A study of 200 patients.

BACKGROUND: Distinct prognostic factors for Wilms tumor (WT) in low- and middle-income countries need identification. METHODS: Retrospective study of patients with WT managed by the International Society of Pediatric Oncology (SIOP) approach for over 11 years (2005-2016) at a single center in Chandigarh, India. RESULTS: The study included 200 patients (median age: 33.5 months). The tumor stage (SIOP) distribution included stage I (30%), II (36%), III (14%), IV (17%), and V (3%). The histology-risk groups were low (8%), intermediate (84%), and high risk (9%). At diagnosis, 68 out of 190 (36%) patients were underweight. The median tumor volume at diagnosis was 481 ml (interquartile ratio [IQR]: 306.9, 686.8, n = 146). Following neoadjuvant chemotherapy, it reduced to 110 ml (IQR: 151.2, 222, n = 77). Treatment was abandoned in 20.5% of the patients. Treatment-related mortality occurred in 13 of 179 (7.2%) patients. Relapse occurred in 26 of 158 (16.5%) patients. The 3-year overall survival (OS) and event-free survival (EFS) of patients who completed therapy were 78.3 and 72%, respectively. The stage (p = .013) and histology (p = .023) influenced OS. A lower OS in stage II (75.4%) versus stage III disease (83.7%) suggested understaging. Patients with a higher tumor volume at diagnosis (p = .005; odds ratio [OR]: 0.99; 95% confidence interval [CI]: 0.99-1.00) or a lower weight-for-age z-score (p = .002; OR: 1.68; 95% CI: 1.21-2.33) had an increased risk of death or relapse. CONCLUSIONS: The 3-year OS and EFS of children who completed therapy were 78.3 and 72%, respectively. A higher tumor volume and lower weight-for-age z-score at diagnosis were identified as distinct adverse prognostic factors. A likely suboptimal lymph node assessment (intraoperative and histopathology) contributed to the understaging of stage III to II disease and reduced survival.

Outcome of Patients with Antenatally Diagnosed hydronephrosis with Respect to Postnatal Diagnosis and Need for Surgical Intervention.

Aims: This study aims to determine the etiology of antenatal hydronephrosis (ANH) and predict need for surgical intervention based on antenatal renal pelvis anteroposterior diameter (APD). Materials and Methods: Combined prospective and retrospective study (2012-2018) of ANH cases with postnatal follow-up. Surgical intervention was correlated with the degree of hydronephrosis (HDN) and pelvis APD measured at the 2nd trimester, 3rd trimester, and postnatal 6-week follow-up. Results: One hundred and sixty-five patients were studied with a total of 219 ANH units. Transient HDN was seen in 116 units. Surgical intervention included pyeloplasty (n = 76), sub ureteric dextranomer injection (n = 8) and nephrectomy (n = 1). Chances of requiring surgery based on the degree of HDN in 2nd and 3rd trimester respectively were mild: 11.32% and 9.52%, moderate: 34.21% and 37.03% and severe: 85.71% and 86.27%. The mean increase in APD between the 2nd and 3rd trimester (n = 50) was lesser in conservatively managed (3.548 ± 4.219 mm) than surgically managed (8.261 ± 5.857 mm) patients (P = 0.002). In another subset (n = 37), the mean increase in APD between the 2nd trimester and postnatal period was less in conservatively managed (1.432 ± 0.612 mm) (P = 0.088) than surgically managed patients (12.91 ± 3.247 mm) (P = 0.004). The area under the receiver operating characteristic (ROC) curve showed that an APD of 8.2 mm in the 2nd trimester and 12.85 mm in the 3rd trimester correlated with the requirement of postnatal surgery. Conclusion: Apart from the degree of HDN, significant changes in APD between 2nd and 3rd trimester and cut off value suggested by the ROC curve will help during antenatal counseling with regard to requirement of postnatal surgery.

Evolution of Investigating Informed Assent Discussions about CPR in Seriously Ill Patients.

CONTEXT: Outcomes after cardiopulmonary resuscitation (CPR) remain poor. We have spent 10 years investigating an "informed assent" (IA) approach to discussing CPR with chronically ill patients/families. IA is a discussion framework whereby patients extremely unlikely to benefit from CPR are informed that unless they disagree, CPR will not be performed because it will not help achieve their goals, thus removing the burden of decision-making from the patient/family, while they retain an opportunity to disagree. OBJECTIVES: Determine the acceptability and efficacy of IA discussions about CPR with older chronically ill patients/families. METHODS: This multi-site research occurred in three stages. Stage I determined acceptability of the intervention through focus groups of patients with advanced COPD or malignancy, family members, and physicians. Stage II was an ambulatory pilot randomized controlled trial (RCT) of the IA discussion. Stage III is an ongoing phase 2 RCT of IA versus attention control in in patients with advanced chronic illness. RESULTS: Our qualitative work found the IA approach was acceptable to most patients, families, and physicians. The pilot RCT demonstrated feasibility and showed an increase in participants in the intervention group changing from "full code" to "do not resuscitate" within two weeks after the intervention. However, Stages I and II found that IA is best suited to inpatients. Our phase 2 RCT in older hospitalized seriously ill patients is ongoing; results are pending. CONCLUSIONS: IA is a feasible and reasonable approach to CPR discussions in selected patient populations.

Outcome Analysis of Reduction and Nonreduction Dismembered Pyeloplasty in Ureteropelvic Junction Obstruction: A Randomized, Prospective, Comparative Study.

Objectives: The objective of this study is to compare the changes in renal function and drainage following open dismembered pyeloplasty with and without renal pelvis reduction. Materials and Methods: Randomized prospective study of children with ureteropelvic junction obstruction undergoing pyeloplasty with (Group 1) and without (Group 2) pelvis reduction over an 18-month period. Postoperative function and drainage were assessed by ethylene dicysteine (EC) scan and intravenous urography (IVU) and renal pelvis size by ultrasonography. Results: Forty-two patients (2 months-11 years) participated. The mean preoperative EC scan function was Group 1: 45.88% ± 14.42% (5%-80%) and Group 2: 39.22% ± 9.75% (21%-53%). (P = 0.117). The mean postoperative EC scan function of Group 1 was 42.64% ± 9.62% (17%-54%) and 43.75% ± 9.88% (17%-58%) and of Group 2 was 44.77% ± 12.82% (20%-68%) and 42.25% ± 8.56% (23%-58%) at 3 months (P = 0.584) and ≥ 1year (P = 0.385), respectively, with no significant difference. None required re-do pyeloplasty. The number of patients with slow drainage, especially at 3 months and also at ≥1-year postoperative period on EC scan was slightly higher in Group 2 compared to Group 1 but did not attain statistical significance. There was postoperative improvement in function and drainage on IVU with no significant difference between the two groups, (P = 0.214; P = 0.99, respectively). At a mean follow-up of 45.5 months, Group 2 also showed significant reduction in pelvis size on ultrasound (P = 0.011). Conclusion: Postoperative function remained stable in both groups. More number of patients achieved unobstructed drainage by 3 mo postoperative after reduction pyeloplasty but drainage patterns were mostly similar between reduction and nonreduction of pelvis group in late follow-up.