Randomized controlled trial to evaluate the rate of successful neonatal endotracheal intubation performed with a stylet versus without a stylet.

INTRODUCTION: Neonates in intensive care units often require endotracheal intubation and mechanical ventilation. During this intubation procedure, a stylet is frequently used along with an endotracheal tube. Despite the widespread use of a stylet, it is still not known whether its use increases the intubation success rate. This study examined the association between stylet use and the intubation success rate in surgical neonates. METHODOLOGY: This single-center study was conducted between December 2021 and December 2022 in the Neonatal surgical intensive care unit of a tertiary care center in Northern India. Infants were randomized to have the endotracheal intubation procedure performed using either an endotracheal tube alone or with a stylet. The primary outcome of the study was to assess the successful first-attempt neonatal endotracheal intubation rate with and without using a stylet. Apart from the rate of successful intubation, the duration of the intubation and complications during the intubation procedures as measured by bradycardia, desaturation episodes, and local trauma were also recorded. Both groups were thus compared on above mentioned outcomes. RESULTS: The total number of neonates enrolled were 200, and the overall success rate (81% in the stylet group vs. 73% in the non-stylet group) was not statistically significant. Intubation time was however less, when stylet was used (16.2 ± 4.3 vs. 17.5 ± 5.0 s, p = .046). When the endotracheal tube size was 3 or less, the success rate was substantially higher in the stylet group (80%) than the non-stylet group (63%), p = .03. No statistical difference was recorded for bleeding and local trauma, though the esophageal intubation rate was higher when intubation was attempted without the stylet. CONCLUSION: Endotracheal intubation using a stylet did not significantly improve the success rate of the procedure, however, intubation time significantly varied between groups and in different conditions. The rigidity and curvature provided by the stylet may facilitate the process of intubation when smaller caliber endotracheal tubes are used.

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.

Delphi Method Analysis and Consensus of Prevalent Distinctive Practices for Biliary Atresia Management after Kasai Portoenterostomy.

Background: Extrahepatic biliary atresia (BA) is seen in infants, with an incidence of 1 in 15,000 live births. The presentation is progressive jaundice, dark-colored urine, and clay-colored stools. Kasai portoenterostomy (KPE) is the commonly performed surgical procedure in these patients. Postoperatively, phenobarbitone, ursodeoxycholic acid (UDCA), steroids, and other drugs are given to improve bile drainage and prevent inflammation and fibrosis. However, a definitive protocol regarding the need for different drugs, dosage, and duration varies across individual surgeons and centers. No universally accepted protocol exists for postoperative management after KPE. Aim: The aim of this study was to know the prevailing postoperative management of BA by subject experts and use the Delphi process to know if the experts want to change their practice based on the results from the survey. Material and Methods: A questionnaire was made after discussing with two experts in the field of BA. The questionnaire was mailed to 25 subject experts. The first survey data were analyzed and shared with all responders. In the second survey, change in the management based on the results from the first survey was assessed. Results: The Delphi questionnaire was answered by 17 experts. Postoperatively, prophylactic antibiotics are prescribed for 6-12 weeks by around 40% and >12 weeks by 30% of respondents. Phenobarbitone is prescribed for <3 months by nearly 50%. UDCA is prescribed for <3 months, ≤6 months, and 6 months-1 year by 47.1%, 23.5%, and 23.5% responders, respectively. Nearly 50% prescribe steroids (mostly prednisolone), and among them, two-thirds prescribe it for 6-12 weeks. Approximately 60% give antiviral drugs to children who are cytomegalovirus immunoglobulin M positive. In our survey, 50% of experts perform 5-10 KPE per year, and 25% each perform 10-15 and >15 KPE per year. The second survey noted that a significant percentage of responders want to change their practice according to consensus. Conclusion: From our Delphi survey, an overview of the postoperative management of BA could be made. However, multicentric studies are required for uniform protocol on the postoperative management of BA.

Anesthetic Management of a Massive Cystic Hygroma of the Neck in a Neonate.

Cystic hygroma of the neck, a congenital benign tumor of the lymphatic system, is a potential cause of neonatal airway obstruction leading to stridor. Meticulous airway evaluation, case appropriate preparation, and use of advanced technology, including videolaryngoscope and ultrasonography, can facilitate the safe management of the difficult airway.

Colonic Atresia Associated with Biliary Atresia.

Colonic atresia (CA) is an uncommon type of intestinal atresia commonly associated with other anomalies, while biliary atresia (BA) is also rare but usually an isolated anomaly. The pathogenesis for either of the anomalies is unclear. The co-occurrence of both pathologies has not been mentioned in the literature. We here discuss the management of CA with BA and the review of pertinent literature.

Tracheoesophageal Fistula with Bilateral Facial Cleft: A Rare Occurrence.

Commissural or lateral facial cleft (macrosomia), classified at Tessier number 7 craniofacial clefts, is a rare congenital anomaly usually associated with deformities of other structures developed from the first and second branchial arches. It affects the esthetics and functional aspect of the oral cavity. Bilateral transverse cleft occurring alone is uncommon and it's with tracheoesophageal fistula (TEF) has not been reported to the best of our knowledge. We report a case of esophageal atresia (EA) and TEF with macrosomia. EA was repaired, and the patient was discharged on full feeds. He is awaiting cleft repair.

A Randomized Control Trial to Evaluate the Effect of Local Instillation of Mitomycin-C at the Porta after Kasai Portoenterostomy in Patients of Biliary Atresia.

Background: Kasai portoenterostomy (KPE) is the initial treatment for biliary atresia (BA). Even after initial jaundice clearance, a significant number of children presented with the reappearance of symptoms due to ongoing fibrosis involving porta and intrahepatic ducts. Mitomycin-C (MMC) is an antifibrotic agent, and the study hypothesized that local application of MMC at porta can decrease fibrosis, which can improve jaundice clearance and lead to better native liver survival (NLS). Materials and Methods: This prospective randomized control trial included children with BA, who were allocated to groups A or B. The patients in both groups underwent standard KPE; in addition, a 5 French infant feeding tube (IFT) was placed near the porta through the Roux limb in Group B children. During the postoperative period, MMC was locally instilled over the porta in Group B children through IFT. Postoperative jaundice clearance and NLS were assessed and compared. Results: A total of 27 children were enrolled in the study, 16 in Group A and 11 in Group B. Both groups were comparable preoperatively. Although the NLS was not statistically significant in Group B, the survival was quite higher, that was 91%, 81%, and 73% at 6 months, 1 year, and 2 years, respectively, compared to 63%, 50%, and 38% in Group A. Conclusion: Children in Group B clinically showed an early jaundice clearance and a better trend of serial bilirubin levels as well as longer NLS than Group A, but it was not statistically significant. The procedure was technically easy, and no complication was encountered related to surgical technique or MMC instillation.

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.

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.

Abdominal Wall Yolk Sac Tumor in a Child.

Midline vascular abdominal wall lesions are likely to be mistaken for vascular malformations in young children. We report a case of large yolk sac tumor located in the anterior abdominal wall just below xiphisternum in a 20-month-old girl diagnosed by raised serum alpha fetoprotein levels and fine-needle aspiration cytology. Preoperative chemotherapy helped in reducing its size allowing wide resection and primary wound closure. This case is reported for the unusual location and role of chemotherapy in management.

Congenital Parotid Fistula: Surgical Management in Two Infants and Review of Literature.

Congenital salivary gland fistulas are uncommon. They develop as a result of abnormalities of the first and second branchial arches. Operative and nonoperative methods of management have been described. We report two rare cases of congenital parotid fistula presenting to us in infancy that were managed surgically.

Benign Renal Tumors in Pediatric Age Group: Retrospective Analysis.

BACKGROUND: Benign renal tumors are extremely rare and were studied here. This series also includes a renal teratoma in a horseshoe kidney, probably only the second in the pediatric literature. MATERIALS AND METHODS: Retrospective review of children with benign renal tumors operated between 2006 and 2018 at one center. RESULTS: Twelve patients (M:F ratio 10:2), age range 3 weeks (31-week gestation) to 13 years presented with large palpable renal swelling (n = 12) and hematuria (n = 3). Computed tomography (CT) scan showed features typical of the tumor. Final histopathology (age group [mean]) showed: multilocular cystic nephroma (MLCN) - n = 5 (41.7%), (11-16 months [13.6]); congenital mesoblastic nephroma (CMN) - n = 4 (33.3%) (classic 1, cellular 3) (0.75-5 months [2.125]); mature cystic teratoma - n = 1 (8.3%): (48 months, in a horseshoe kidney), and angiomyolipoma (AML) - n = 2 (16.7%) (144 months [sporadic] and 156 months [tuberous sclerosis]) One patient with cystic teratoma with no calcification on CT scan received pre-operative chemotherapy as fine-needle aspiration cytology (FNAC) reported malignant small blue cell tumor. Nephroureterectomy with Gerota's fascia could be done easily in all without intraoperative complications. Delay in presentation in MLCN and CMN led to increased symptoms and CT scan changes. All patients did well in 1.5-12 years (median 3 years) follow-up including cellular mesoblastic nephroma. CONCLUSIONS: Benign renal tumors often occur in specific age groups but may overlap that of Wilms tumor. Proper interpretation of clinical presentation, CT scan, and FNAC findings help in avoiding preoperative chemotherapy. Upfront nephroureterectomy is curative. Histopathological findings decide further treatment. Children with AML and tuberous sclerosis need lifelong follow-up.

Type IV Congenital Pouch Colon in Male Children: Anatomical Variations and a Proposed New Subclassification.

BACKGROUND: Congenital pouch colon (CPC) is a rare variant of anorectal malformation. In male patients, CPC communicates distally with the urogenital tract by a large fistula. The CPC cases which do not fulfill the criteria as mentioned in the classical description are reported along with the pertinent literature review. MATERIALS AND METHODS: This was a retrospective study from January 2004 to December 2017 of male children with Type IV CPC. We evaluated clinical presentation, primary management, anatomical relationship, previous surgical intervention, definitive management, result, and outcome in terms of continence status on Templeton score. RESULTS: Fifty-one children were included in the study among whom 36 children (Group 1) had a colovesical fistula and 15 children (Group 2) had no communication of the pouch with the genitourinary tract. In Group 2 children, the clinical presentations and management were varied: 4 underwent primary pull-through procedure, whereas 11 underwent staged procedure. Group 2 included three children in whom a narrow and thin-walled anal canal or anal canal with lower rectum was present, which was incorporated during the pull-through procedure. On continence assessment, only one child in Group 1 had "good" continence score compared to four children (three having anal canal) in Group 2. CONCLUSION: CPC Type IV can present without genitourinary tract communication (fistula), contrary to its emblematic description. Awareness about anatomical variations and adaptation of surgical technique accordingly is vital. The identification of the anal canal with or without the lower part of the rectum (even though apparently narrow and thin walled) and incorporation of this in bowel continuity lead to better outcomes in terms of continence.

Clinical Presentation and Surgical Management of Neonatal Tumors: Retrospective Analysis.

AIMS: Neonatal tumors (NTs) include a group of diverse neoplasms. In this study, we reviewed our data for clinical presentations, management options, and outcome. MATERIALS AND METHODS: All patients from 0- to 1-month age presenting with solid tumors, from 2006 to 2018 were studied. The gender, presentation, location, type of tumor, and management were analyzed. The final diagnosis was made with histopathology in all cases. Hemangiomas and lymphangiomas were excluded from the study. RESULTS: A total of 32 neonates were studied. The most common tumor was sacrococcygeal teratoma (SCT,16) followed by teratoma at other sites including two cases of fetus-in-fetu, soft-tissue sarcoma (STS, 4), mesenchymal hamartoma (2), hemangioendothelioma (2), and other rare tumors. Three tumors were diagnosed antenatally; of whom, two were neither visible externally nor palpable. Complete surgical excision was done for all except in a case of ovarian cyst where near-total cystectomy was done. No patient received chemotherapy or radiotherapy. Six patients had postoperative complications, including two who had local recurrence requiring excision. There was one mortality. All the other patients are doing well during follow-up. CONCLUSION: NTs have varied presentations. SCT and STS were the most common benign and malignant tumor, respectively. Early diagnosis and complete surgical excision are often curative for all, regardless of the pathology with the minimal role of chemotherapy or radiotherapy.

Evaluation of Serum Levels of Trace Elements in Children with Biliary Atresia and their Correlation with Liver Histopathology.

BACKGROUND: Children with biliary atresia (BA) have impaired metabolism of trace elements (TEs) (i.e., zinc, copper, selenium, and manganese), leading to an alteration in the serum levels. However, this alteration in serum level has any correlation with liver histopathological changes is not yet clear. MATERIALS AND METHODS: This prospective study attempts to evaluate the preoperative serum levels of TE in comparison to controls and its correlation with liver histology in children with BA. Kasai portoenterostomy (KPE) and liver biopsy were performed in all cases. On liver histology, various parameters assessed and were graded according to predefined criteria. Serum levels of TE were determined again 12 weeks post-KPE and compared with the preoperative levels. RESULTS: Mean(±standard deviation [SD]) preoperative serum Zn, Cu, Se, and Mn levels (in µg/dl) in BA patients were 41.6 ± 12.8, 130.6 ± 12.8, 50.0 ± 10.0, and 32.0 ± 20.0, respectively; in controls, these levels were 77.9 ± 13.7, 133.7 ± 13.7, 87.0 ± 13.0, and 8.0 ± 5.5, respectively. Mean postoperative levels in all patients were 68.5 ± 19.0, 91.7 ± 19.0, 79.0 ± 19.0, and 28.0 ± 12.0, respectively. Mean(±SD) postoperative serum Zn, Cu, Se, and Mn levels in BA patients with bile excretion were 73.8 ± 14.9, 83.6 ± 13.8, 85.0 ± 15.0, and 26.0 ± 10.0, respectively, whereas in those with no bile excretion, they were 40.6 ± 12.8, 134.0 ± 23.0, 49.0 ± 11.0, and 44.0 ± 16.0, respectively. In liver histology, specific parameters showed correlation with high Mn and low Zn levels. CONCLUSION: Serum TE levels are altered in children with BA and the establishment of successful biliary drainage may change the subsequent postoperative serum concentration. Serum Zn and Mn levels can signify specific histopathological liver changes and the extent of liver damage.

Nutritional Status of Children with Biliary Atresia and the Role of Portoenterostomy to Improve Nutrition.

BACKGROUND: Nutritional depletion and growth stunting are present in patients with biliary atresia; "normal" nutrient and vitamin supplementation fail to correct these deficiencies. Children with this condition form the largest group for possible liver transplantation in the future; hence, stress should be laid on close attention to their nutrition. METHODS: Twenty-five patients with biliary atresia as cases and 25 age-matched children as controls were enrolled in the study from November 2010 to June 2012. Preoperatively, patients underwent standard investigations and anthropometric measurement (weight, height, and head circumference) assessment. Nutritional status (assessed with standard growth chart) was compared with control population, and children were divided into poor nutritional status and good nutritional status. Kasai's portoenterostomy was performed in all patients, and comparison was done between preoperative nutritional status with postoperative status of children and also between hepatic iminodiacetic acid (HIDA) scan-positive (patent bilioenteric pathway) children with HIDA scan-negative children. Postoperatively, after 12 weeks, the same anthropometric measurements were taken again, growth velocity (GV) was assessed, and children were divided into poor, average, and good GV. RESULTS: Nutritional status of children with biliary atresia was significantly poor than that of control group. Postoperatively, children had better nutritional status than preoperative nutritional status, especially in HIDA scan-positive children. GV was also significantly better in those children in whom postoperative HIDA scan was positive. CONCLUSION: Children with biliary atresia have poor nutritional status in comparison to normal population and require multifaceted approach to achieve adequate nutrition. Establishment of a patent bilioenteric pathway in these children improves their nutritional status and GV.

Palatoglossal fusion with cleft palate and hypoplasia of cerebellar vermis.

A new-born male presented within 12 h of birth with respiratory distress. On examination and workup, he had palatoglossal fusion, cleft palate and hypoplasia of the cerebellar vermis. A 2.5 Fr endotracheal tube was inserted into the pharynx through nostril as a nasopharyngeal stent, following which his respiratory distress improved. Once child was optimised, then feeding was started by nasogastric tube and feeds were tolerated well. Elective tracheostomy and gastrostomy were done, followed by release of adhesions between the tongue and palate at a later stage. Review of literature suggests that palatoglossal fusion is uncommon and presents as an emergency. Mostly, these oral synechiae are associated with digital and/or cardiac anomaly. Other disorders associated with intra-oral synechiae include congenital alveolar synechiae, van der Woude syndrome, popliteal pterygium syndrome and oromandibular limb hypogenesis syndrome. The authors report a hitherto undescribed association of palatoglossal fusion with cleft palate and hypoplasia of the cerebellar vermis.

Ruptured rectal duplication with urogenital abnormality: Unusual presentation.

Rectal duplication (RD) accounts for 5% of alimentary tract duplication. A varied presentation and associated anomalies have been described in the literature. Antenatal rupture of the RD is very rare. We present an unusual case of a ruptured RD associated with urogenital abnormalities in newborn male. We are discussing diagnosis, embryology, management and literature review of ruptured RD.

Crossed fused renal ectopia: Challenges in diagnosis and management.

AIM: Crossed fused renal ectopia is a rare congenital malformation, which is reported to be usually asymptomatic but may have varied presentations. This survey was conducted to study the clinical profile and the challenges posed in the management of this entity. MATERIALS AND METHODS: Retrospective analysis of 6 patients diagnosed to have crossed fused renal ectopia during 1997-2010. The diagnosis was confirmed during surgical exploration in one patient. In one patient it was detected on antenatal ultrasonography and in the other 4 patients it was detected during investigations for abdominal pain, abdominal mass, anorectal malformation and urinary tract infection. RESULTS: The left moiety was crossed and fused with the right moiety in 4 cases. Ultrasonography was found to be a good screening investigation with useful diagnostic contributions from CT scans, radionuclide scintigraphy and magnetic resonance urography. Micturating cystourethrography revealed presence of VUR in 4 cases, 3 of whom have undergone ureteric reimplantation. Two patients required pyeloplasty for pelviureteric junction obstruction; in one of these patients the upper ureter was entrapped in the isthmus. In one patient, a non-functioning moiety resulted in nephrectomy. All children were asymptomatic at last follow-up with stable renal functions. CONCLUSIONS: Crossed fused renal ectopia was detected in most patients during investigation for other problems. It was found more commonly in boys. The left moiety was crossed to the right in the majority of cases. Associated urological problems were found in most cases and required the appropriate surgical management.