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.

MYCN amplification, TERT rearrangements and ATRX mutations in neuroblastoma: clinicopathological correlates- an Indian perspective.

BACKGROUND: Neuroblastoma (NB) is the most common extracranial solid tumour in childhood with a diverse clinical presentation and course. The early age of onset, high frequency of metastatic disease at diagnosis and tendency for spontaneous regression in infancy sets it apart from other childhood tumors. This heterogeneity is largely attributed to underlying genetic aberrations which are distinct in low-risk and high-risk NB. To this end, we sought to analyse our NB cases for the molecular alterations and find its correlation with clinical behaviour. METHODS: NB cases (n = 50) diagnosed over last 7 years were retrospectively analysed for MYCN amplification (fluorescent-in-situ hybridization), TERT rearrangements (qRT-PCR), ATRX mutations (immunohistochemistry). These findings were correlated with demographic profiles, histologic features and clinical outcome. RESULTS: Age ranged from 1 month to 30 years (mean 2.8 years) with male preponderance. Poorly differentiated subtype constituted the majority (64%), followed by differentiating (28%) and undifferentiated subtype (8%) which were equally distributed across all age groups. MYCN amplification, TERT-mRNA upregulation and ATRX mutations was observed in 30%, 42% and 24%, respectively. Cases with TERT-mRNA upregulation were distributed equally across all histological subtypes while those with ATRX mutations and MYCN amplification were frequent in poorly differentiated NB. ATRX mutation was mutually exclusive of TERT-mRNA upregulation and MYCN amplification. Kaplan-Meier analysis revealed significantly shorter overall and progression-free survival for tumors harboring MYCN amplification and TERT-mRNA upregulation, while that for ATRX mutant tumors was not significant. CONCLUSIONS: Our results provide data indicating poor clinical outcome in NB carrying MYCN amplification and TERT-mRNA upregulation.

Mucoepidermoid carcinoma of the trachea in a 9-year-old male child: case report and review of literature.

Mucoepidermoid carcinoma (MEC) is most common malignancy of minor salivary glands in adults. Pulmonary MEC is extremely uncommon comprising of only 0.1%-0.2% of the primary lung malignancies and <1% of primary bronchial tumors. It is even rarer in children and literature limited to few case reports only. Here we present a case report of a 9-year-old boy diagnosed with primary MEC of trachea along with review of the literature. A 9-year-old male child presented with complaint of dry cough for two years which was later associated with shortness of breath after one year. Bronchoscopic examination revealed a growth arising from right lateral wall of carina occluding 50% of the lumen and detailed histopathological examination revealed it to be a MEC of the trachea. Patient underwent local excision of the tumor with primary anastomosis. In view of positive margins adjuvant radiotherapy of 60 Gy in 30 fractions were given to the tumor bed. Patient tolerated the treatment well and is disease free at 6 months follow-up. Experience with MEC of the trachea in children is limited and optimal treatment protocols have not been defined, with current treatment mainly extrapolated from MEC of the salivary glands.

Teratoid Hepatoblastoma with Multi-Lineage Differentiation: An Uncommon Histological Variant and Review of Literature.

Background: Teratoid hepatoblastoma is an uncommon but well-recognized variant of mixed hepatoblastoma. Case report: A one-year female child presented with palpable and progressively increasing right abdominal mass for 3 months. The contrast-enhancing computed tomography (CECT) abdomen revealed a large heterogeneous hepatic mass measuring 12 × 6.6 × 6 cm. Histopathological examination of the resected specimen showed a mixed hepatoblastoma (epithelial and mesenchymal) with teratoid features and multi-lineage differentiation (all three germ cell layers). A focus showed embryonal rhabdomyosarcomatous element. Conclusion: Teratoid hepatoblastoma can show a wide range of heterologous differentiation that may pose a significant diagnostic dilemma. Such a broad spectrum has not been described in the literature previously. An appropriate immunohistochemical panel may be needed to identify and delineate the various heterologous differentiation to clinch the correct diagnosis. Secondary somatic malignancy such as rhabdomyosarcoma can develop in a teratomatous element.

Postoperative hyponatremia in neonates with esophageal atresia and tracheoesophageal fistula receiving restricted hypotonic fluids.

Background: During the postoperative course following neonatal surgery, several stimuli like respiratory distress, pain, and stress cause the release of the antidiuretic hormone which can induce hyponatremia. This hyponatremia due to syndrome of inappropriate antidiuretic hormone secretion (SIADH) in neonates can lead to neurologic impairment and in severe cases can cause significant morbidity and mortality. Lung involvement in neonates undergoing TEF makes this subset of patients vulnerable to this entity because most of them are sick and require ventilation in the postoperative period. The incidence of postoperative hyponatremia following neonatal surgery has not been studied vastly. To the best of our knowledge, this is the first prospective study that has analyzed the incidence of postoperative hyponatremia in this vulnerable population. Methods: Prospective observational study to assess the incidence of postoperative hyponatremia in neonates with esophageal atresia and tracheoesophageal fistula (EA and TEF) receiving restricted hypotonic fluids. As per the unit policy N/4 5% D is given in the postoperative period. Most neonatal units follow a protocol in which fluid is hiked daily to reach 150 ml/kg/day in 5-7 days. However, in our neonatal surgical unit a protocol to restrict the maintenance fluid at 100 ml/kg/day irrespective of day of life is followed. Results: Out of a total of 90 neonates (270 sodium measurements), we identified 16 with hyponatremia (11%). Most of the neonates had mild hyponatremia(130-135 meq/l). The incidence of moderate and severe hyponatremia was low. Conclusion: Postoperative restriction of fluids especially in neonates who are at a high risk for developing SIADH can lead to decreased incidence of severe hyponatremia.

A Rare Association of Obstructed Hemivagina with Ipsilateral Renal Agenesis with Congenital Pouch Colon.

BACKGROUND: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA), or Herlyn-Werner-Wunderlich syndrome, is a rare Mullerian duct anomaly. Several associations of OHVIRA with other anomalies are being reported. CASE: A 13-year-old girl reported with acute onset abdominal pain. Patient was operated on in the past for type IV congenital pouch colon (all stages complete). She was diagnosed with hematometrocolpos and underwent tube vaginostomy. Further workup was suggestive of OHVIRA, which was subsequently managed with surgical repair. CONCLUSION: Delineation of reproductive anomalies in female patients with anorectal malformations is of paramount importance. OHVIRA syndrome should be considered at a high index of suspicion in female patients with a solitary functioning kidney. Nonspecific symptoms in an adolescent female can lead to erroneous judgment, leading to unnecessary investigations, which, if not well managed in time, can be detrimental to fertility.

Chronic peritoneal dialysis in children with chronic kidney disease: An experience from a North Indian teaching institute.

INTRODUCTION: Chronic peritoneal dialysis (CPD) is an important modality of renal replacement therapy (RRT) in children of all ages with end-stage renal disease (ESRD). We retrospectively assessed the clinical profile of children with chronic kidney disease (CKD) initiated on CPD at a tertiary care centre in Northern India. MATERIALS AND METHODS: Retrospective data of 13 children with CKD and initiated on CPD between 2016 and 2019 were retrieved and analysed. The demographic and clinical profile, aetiology of CKD, method of catheter insertion, mode of dialysis, complications, and catheter survival rate were analysed. RESULTS: The median age at the onset of the symptoms was 81 months interquartile range (IQR 11-90) and the median age at the diagnosis was 81 months (IQR 36-103). The median age at the initiation of CPD was 92.97 months (IQR 74.43-108.79). The median serum creatinine at the initiation of CPD was 6.3 mg/dL (IQR 4.25-8.4). During a total study period of 84 CPD months, we observed 16 catheter-related complications and a complication rate of 1 per 5.25 CPD months. The overall peritonitis rate was 1 episode per 13.66 patient-months (0.87 episodes per patient-year). The catheter displacement/migration was seen in 23% of the cases. The median duration of follow-up was 175 days (IQR 85-249) with the longest follow-up duration of 502 days. CONCLUSION: CPD is the modality of choice for smaller children with ESRD as venous access is difficult to achieve in smaller children. Complications especially related to infections are a major concern in addition to poor growth associated with ESRD.

Role of Cyclin D1 and BCOR Immunohistochemistry in Differentiating Clear Cell Sarcoma of Kidney From its Mimics.

BACKGROUND AND AIM: Clear cell sarcoma of kidney (CCSK) is the second most common pediatric renal malignancy, constituting ∼3% of renal tumors. Due to its morphologic diversity, the diagnosis of CCSK is often challenging. Recent studies have identified internal tandem duplication of BCL6 corepressor (BCOR) gene in CCSKs which coupled with cyclin D1 immunoreactivity, is helpful in differentiating it from its mimics, particularly blastema-rich Wilms tumor (WT), malignant rhabdoid tumor (MRT), and congenital mesoblastic nephroma (CMN). We aimed to evaluate the utility of cyclin D1 and BCOR immunohistochemistry in differentiating CCSK from its morphologic mimics. MATERIALS AND METHODS: Our cohort comprised of 38 pediatric renal tumors which included CCSK (n=18), WT (n=10), MRT (n=5), and CMN (n=5) cases. A detailed clinicopathologic analysis was performed, and tissue microarray were constructed for CCSK and WT, while MRT and CMN tumors were individually stained. RESULTS: The age ranged from 2 months to 16 years with male:female ratio of 3:1. Strong, diffuse nuclear immunoreactivity for cyclin D1 and BCOR was noted in 61% (n=11/18) and 83% (n=15/18) of CCSK, respectively, while it was significantly less in WT (n=3/10 for cyclin D1) (n=2/10 for BCOR). None of the MRT and CMN examples demonstrated any immunoreactivity. Interestingly, only the blastemal component of WTs showed distinct, rare nuclear immunoreactivity for cyclin D1 or BCOR and the combination of these was never positive in a given case. CONCLUSION: Our results provide evidence that concurrent immunopositivity with cyclin D1 and BCOR is helpful in distinguishing CCSK from its morphologic mimics.

Oral melatonin as part of multimodal anxiolysis decreases emergence delirium in children whereas midazolam does not: A randomised, double-blind, placebo-controlled study.

BACKGROUND: Pre-operative anxiety is a risk factor for emergence delirium in children and a multimodal approach including sedatives and nonpharmacological measures is the current strategy to tackle this anxiety. The efficacy of oral melatonin as a component of multimodal anxiolytic strategy to decrease emergence delirium is not well studied. OBJECTIVE: The aim of this study was to evaluate the efficacy of a multimodal anxiolytic strategy including oral melatonin or midazolam to decrease emergence delirium after sevoflurane anaesthesia. DESIGN: A randomised, double-blind, parallel arm, placebo-controlled trial. SETTING: Tertiary care teaching hospital from July 2019 till January 2020. PARTICIPANTS: Children in the age group of 3 to 8 years who received sevoflurane anaesthesia for elective ambulatory procedures. INTERVENTIONS: Children were randomised to receive oral premedication with either melatonin 0.3 mg kg-1, midazolam 0.3 mg kg-1 or honey as placebo. All the children received standardised nonpharmacological measures involving multiple techniques to allay anxiety. The anaesthetic plan was also standardised. MAIN OUTCOME MEASURES: The primary outcome was the incidence of emergence delirium as assessed by the Watcha scale in the postanaesthesia care unit. The secondary outcomes were pre-operative anxiety assessed using a modified Yale Preoperative Anxiety scale, patient compliance with mask induction using the Induction Compliance Checklist and postoperative sedation. RESULTS: Data from 132 children were analysed. Melatonin significantly reduced the incidence of emergence delirium compared to placebo: 27 vs. 50%, respectively, an absolute risk reduction of 23.3 [95% confidence interval 3.7 to 42.9), P = 0.03]. Melatonin also significantly reduced the risk of emergence delirium compared with midazolam: 27 vs. 56%, respectively, an absolute risk reduction of 29.2 (95% CI 9.5 to 48.8). The midazolam group had a similar incidence of emergence delirium as placebo. Sedation scores were similar in the three groups postoperatively. The incidence and score of pre-operative anxiety as well as the compliance with mask induction were similar in the three groups. CONCLUSIONS: A multimodal anxiolytic approach including oral melatonin, as opposed to oral midazolam, significantly reduced emergence delirium after sevoflurane anaesthesia. TRIAL REGISTRATION: CTRI/2019/06/019850 in Clinical Trial Registry of India (www.ctri.nic.in).

Role of feeding jejunostomy in major anastomotic disruptions in esophageal atresia: A pilot study.

AIMS: To investigate the role of feeding jejunostomy (FJ) in patients of esophageal atresia with anastomotic leak (AL) to decrease the degree of gastroesophageal reflux (GER) and its effect on anastomotic healing. MATERIALS AND METHODS: Twenty neonates, with major AL and severe GER after primary repair were managed with decompressing gastrostomy and transgastric FJ and analyzed prospectively. RESULTS: Male to female ratio was 1.7:1. Mean birth weight was 2.2 kg. Anastomotic gap ranged from 0 to 4 cm. The amount of leak was more than 20% of nasogastric feeds. Gastrostomy and FJ was done on an average of the 12(th) postoperative day, after observing the general condition, chest tube output, lung expansion, and ventilatory requirement. There was a drastic reduction in chest tube output and lung expanded in all patients. Average hospital stay was 36 days (8-80 days). Sixty percentage patients were discharged successfully on FJ. Esophagogram demonstrated healing and leak free patency after an average of 1.5 months. GER was noted in seven patients, four developed stricture, and one had pseudodiverticulum in follow-up. CONCLUSION: Decompressing gastrostomy and FJ can be an alternative to managing major ALs. It helps in healing of anastomotic dehiscence and in preserving the native esophagus.

Undiagnosed ureteroinguinal hernia with solitary kidney in a child with ureteric injury during herniotomy.

Iatrogenic ureteric injuries are an uncommon but difficult problem to manage. Although ureteric injury has been reported during hernia surgery in adults, it has not been reported in children during herniotomy. An 18-month-old male child underwent repair of an inguinal hernia and developed postoperative anuria. Investigations revealed that the patient had a solitary kidney and had injury to the solitary ureter. We successfully managed this patient using the vermiform appendix to replace a segment of the injured ureter. The possible mechanism of injury and the relevant literature are discussed.

Gluteal and perianal hidradenitis suppurativa associated with tuberulosis.

Perineal and gluteal hidradenitis suppurativa is a rare, chronic inflammatory disease of the skin caused by concomitant occlusion of the apocrine glands. We present a case report of a young male with no co-morbid conditions in which histopathology and DNA PCR showed association of chronic hyderadenitis suppurativa with tuberculosis. There was relapse after three months of completion of anti tubercular therapy. He was subsequently treated successfully with wide excision, split thickness skin grafting and a defunctioning stoma.

Prophylactic antibiotic usage in patients undergoing inguinal mesh hernioplasty - A clinical study.

BACKGROUND: There is ambiguity about the use of antibiotic prophylaxis in inguinal mesh hernioplasty. We have tried to assess the efficacy of antibiotic prophylaxis in this procedure. MATERIALS AND METHODS: A randomized double blind placebo controlled study was conducted which included 55 patients who underwent an inguinal mesh hernioplasty over a 2 year period. The patients were evaluated for the status of the suture line as well as the presence of wound infection. RESULTS: Out of 55 patients 29 were randomized to the antibiotic arm and 26 to the placebo group. The groups were well matched for all variables studied excluding wound infections, which occurred at a rate of 10.34% (n = 3) in the antibiotic group and 15.38% (n = 4) in the placebo arm, (p > 0.01). CONCLUSION: This study did not document any statistically significant difference observed between those who received antibiotics and those receiving placebo in terms of any of the prognostic end points evaluated for Lichtenstein mesh hernioplasty.