Recurrent tracheo-esophageal fistula- management challenges: Case report and review of current literature.

Recurrent tracheoesophageal fistula is a rare complication of esophageal atresia surgery with an incidence of 3-15%. The presentation is subtle and is often missed, presenting as choking episodes during feed and recurrent chest infections. It is both a diagnostic and management challenge and requires a dedicated multidisciplinary pediatric surgical setup with adequate infrastructure for optimal management. We present a case of recurrent tracheoesophageal fistula which was diagnosed at our center. The patient underwent successful surgical management and is thriving well at six months follow-up period.

Assessing the Feasibility of Predicting Intermediate Outcomes of Esophageal Atresia Using Okamoto Classification: A Prospective Study.

Purpose: Several risk classifications based on various preoperative factors have been proposed to prognosticate the immediate survival of children operated for esophageal atresia. A major drawback of these classifications is that they only focus on immediate survival while ignoring the long-term morbidity and mortality in these children. Our study aims to bridge this gap in knowledge by studying the impact of one such classification (Okamoto's classification) on mortality and morbidity during a period of 1 year after discharge from the hospital in operated cases of esophageal atresia. Materials and Methods: After institutes ethical clearance, 106 children operated for esophageal atresia-tracheoesophageal fistula between 2012 and 2015 were studied prospectively for a period of 1 year after their discharge. The children were graded as per Okamoto classification. The primary objective was to determine the efficacy of this classification in predicting the survival rates in infancy and the secondary objective was to compare the complication rates in these children based on the classification. Results: Sixty-nine children met the inclusion criteria. There were 40, 15, 10, and 4 children in Okamoto Classes I, II, III, and IV, respectively. Twenty-one patients (30%) died during the follow-up period with the maximum number of deaths occurring in Okamoto Class IV (75%) and the minimum in Okamoto Class I (17.5%) (P = 0.003). There was a significant correlation between the Okamoto classes with the incidence of poor weight gain (P = 0.001), lower respiratory tract infection (P = 0.007), and failure to thrive (P = 0.01) higher in Okamoto IV and III as compared to I and II. Conclusion: Okamoto prognostic classification during the initial hospitalization is relevant even at 1 year follow-up with increased mortality and morbidity in Okamoto Class IV as compared to Class I.

Quantification of the Androgen and Estrogen Receptors in the Penile Tissues of Hypospadias in Comparison with Normal Children.

INTRODUCTION: Hypospadias is a common congenital abnormality typified by a proximally placed ectopic urethral meatus along the ventral surface of the penis. Androgen receptor (AR) and estrogen receptor (ER) expression in the hypospadias tissues may be altered in hypospadias. METHODOLOGY: We evaluated by immunohistochemistry the AR and ER expression in 75 tissues from hypospadias repair, and compared this expression to that of tissue from 75 patients undergoing circumcision. We also compared the intensity of AR and ER expression between different severities of hypospadias. RESULTS: AR quantitative grading score decreased with severity of hypospadias, while the ER score increased as the hypospadias worsened, which was statistically significant (p-value <0.05). CONCLUSION: The penile tissue AR expression is decreased and ER expression is increased with increasing severity of hypospadias.

A prospective observational study to evaluate the change in inhibin-B as a marker of sertoli cell function in children subjected to surgical correction for undescended testes.

Background and Objectives: Undescended testes (UDT) or cryptorchidism is a common congenital disability characterised by the absence of at least one testicle from the scrotum. The primary aim of surgical correction is to preserve fertility potential and prevent complications including trauma, malignancy, hernia and torsion. Often, children, especially in developing countries, present late with UDT. The effect of surgical correction in the recovery of Sertoli cell function in children aged more than 2 years is not apparent. The present study was conducted to study the change in inhibin-B level as a marker of Sertoli cell function in surgically corrected UDT in a heterogeneous population. Materials and Methods: A prospective observational study conducted over a 3-year period at a tertiary care paediatric surgery centre recruited 76 children with UDT undergoing surgical correction. Inhibin-B as a marker for Sertoli cell function was studied preoperatively and postoperatively. Continuous variables were summarised by calculating mean, standard deviation, median and interquartile range (IQR). Quantile versus quantile plotting was done to assess the distribution of the data. Data were analysed in two groups, with participants aged <2 years (Group A) and more than 2 years (Group B). Wilcoxon signed-rank test was used to compare the pre-operative and post-operative value. Results: In Group A (n = 39), the median (IQR) of pre-operative inhibin-B was 181 pg/ml (148-254) and post-operative inhibin-B was 230 pg/ml (176-296). In Group B, the median (IQR) of pre-operative inhibin was 70 pg/ml (44-104) and post-operative inhibin was 102 pg/ml (46-176). There was a significant increase in post-operative inhibin when compared to the pre-operative inhibin (P = 0.015 and 0.012, respectively, in Group A and B). Luteinizing hormone (LH) showed a significant decrease (P = 0.002) in Group A following surgery but bordering on significance in Group B (P = 0.43). On the other hand, follicle-stimulating hormone showed a significant decrease (P < 0.01) in Group B following surgery but not in Group A (P = 0.87). Conclusion: The mean post-operative inhibin-B levels were increased significantly as compared to the pre-operative levels indicating either a successful orchiopexy/adequate germ cell number or both. The benefit of orchiopexy may extend even to children presenting late for evaluation.

Acupuncture-D" - Bilateral Pneumothoraces Following Dry Needling.

Presentation A 24-year-old newly graduated junior doctor presented to the emergency department with acute onset chest pain, haemoptysis and exertional dyspnoea following a dry needling session. Diagnosis Chest x-ray showed bilateral pneumothoraces, worse on the right side. Treatment The bilateral pneumothoraces were treated conservatively with supplemental oxygen initially. On the second day of admission, repeat chest x-ray demonstrated a worsening right sided pneumothorax. While vitally stable, the patient however had become increasingly dyspnoeic, and a needle aspiration was performed on the right side with good effect. Conclusion The anatomical location targeted along with the patients low-normal BMI makes her high-risk when considering the skin-to-pleura distance. Although the incidence of pneumothorax is low, it is imperative that we improve awareness both for the treating physician and the diagnosing clinician. We must begin to fill the distinct lack in available literature surrounding the potential adverse effects of dry needling.

Intracranial aneurysms: The era of endovascular intervention in Australia.

Intracranial aneurysms (ICAs) can be treated by surgical or endovascular techniques. Endovascular procedures have become common in many specialties, including neurosurgery. In this paper we aim to examine the overall numbers and trends in treatment of IA in Australia. This study was a ten-year retrospective analysis of the Medicare Benefits Schedule (MBS) and Australian Institute of Health and Welfare (AIHW) National Hospital Morbidity databases. These databases were searched for codes corresponding to clipping of ICAs and endovascular coiling of ICAs from the financial years 2008-2009 to 2018-2019. The total number of procedures increased from 2.25 to 3.37 per 100,000 from 2008 to 2009 to 2018-2019 financial years in the MBS database and 7.90 to 10.35 per 100,000 people in the AIHW database for the same financial years. The rate of change of MBS was 0.12 (0.08- 0.16, p < 0.0001) and for AIHW 0.23 (0.19- 0.28, p < 0.0001). Endovascular coiling reached parity with surgical clipping in the 2010-2011 financial year. Women were over twice as likely as men to be treated. The frequency of treatment per 100,000 people peaked in the 55-64 year-old age group. Tasmania performed the most number of treatments per capita compared to other Australian states and territories, according to the MBS database. These figures from Australian databases are in line with other countries from studies around the world that examine preferences of intervention and demographics.

When Fryn met Edward: Two rare syndromes in a single patient.

A neonate born at our centre was diagnosed as Fryns Syndrome ie congenital diaphragmatic hernia with facial dysmorphism and distal limb anomalies, which is a rare disorder with only a few hundred cases reported till date.With high clinical index of suspicion and further evaluation, the diagnosis was confirmed. The baby was initially stabilized and later underwent repair of the diaphragmatic hernia. Despite best measures, the baby could not be salvaged. When severe, this can be lethal and diagnosis can only be made after autopsy. However, with early suspicion, better modalities of investigations available and improved NICU care, these babies can be salvaged. We report a case of Fryns Syndrome who was incidentally found to have Edward Syndrome as well. Such an extremely rare combination is yet to be reported in medical literature.Also with updated genetic studies, better diagnostics and treatment options coming up in future, there are chances to improve the survivability of these babies. It is prudent to document all such cases to aid in better understanding of the disease process.

Antenatally Detected Functional Adrenocortical Adenoma: A Case Report and Review of Current Literature.

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

Use of therapeutic caffeine in acute care postoperative and critical care settings: a scoping review.

BACKGROUND: Caffeine is the most utilised psychoactive drug worldwide. However, caffeine withdrawal and the therapeutic use of caffeine in intensive care and in the perioperative period have not been well summarised. Our objective was to conduct a scoping review of caffeine withdrawal and use in the intensive care unit (ICU) and postoperative patients. METHODS: PubMed, Embase, CINAHL Complete, Scopus and Web of Science were systematically searched for studies investigating the effects of caffeine withdrawal or administration in ICU patients and in the perioperative period. Areas of recent systematic review such as pain or post-dural puncture headache were not included in this review. Studies were limited to adults. RESULTS: Of 2268 articles screened, 26 were included and grouped into two themes of caffeine use in in the perioperative period and in the ICU. Caffeine withdrawal in the postoperative period increases the incidence of headache, which can be effectively treated prophylactically with perioperative caffeine. There were no studies investigating caffeine withdrawal or effect on sleep wake cycles, daytime somnolence, or delirium in the intensive care setting. Administration of caffeine results in faster emergence from sedation and anaesthesia, particularly in individuals who are at high risk of post-extubation complications. There has only been one study investigating caffeine administration to facilitate post-anaesthetic emergence in ICU. Caffeine administration appears to be safe in moderate doses in the perioperative period and in the intensive care setting. CONCLUSIONS: Although caffeine is widely used, there is a paucity of studies investigating withdrawal or therapeutic effects in patients admitted to ICU and further novel studies are a priority.

Common cloaca: An uncommon entity posing diagnostic and management challenges: Our experience with nine cases.

BACKGROUND: Cloacal anomalies are the severest and most complex of all anorectal malformations (ARMs). They require careful evaluation and meticulous surgery tailored to suit each variant. We present our experience in a series of nine cases. METHODS: This includes a retrospective review of 9 cases of cloaca managed at a tertiary care centre between 2015 and 2019. RESULTS: Associated anomalies were seen in 44% cases. The definitive surgery was performed at a mean age of 15.2 months (10 months-19 months), the definitive surgery being rectal separation with total urogenital mobilisation. The common channel as measured during panendoscopy was up to 3 cm in 7 patients (78%), and only 2 patients had a common channel of more than 3 cm (22%). Of the 34 procedures that these nine patients underwent, there were four complications (12%). The median follow-up period after stoma closure was 18 months (5-32 months), and the mean age at last follow-up was 38 months (22-48 months). Five children (63%) had spontaneous voiding and remained dry in the intervening period. Three patients (37%) had poor urinary stream with dribbling and high postvoid residue requiring clean intermittent catheterisation. Six patients had faecal soiling (66%); four had daily soiling; and two had occasional soiling. Four patients had constipation (44%). Seven patients (77%) required daily enemas for bowel evacuation and to remain dry. CONCLUSION: Cloacal anomalies are rare and complex ARMs. Satisfactory urinary and bowel continence rates can be achieved even in these complex anomalies.

Klippel Feil syndrome with crossed fused renal ectopia with pelviureteric junction obstruction: A rare association.

An 11-year-old female patient presented with congenital spinal deformity with a history of occasional pain in the right flank for a duration of six months. On evaluation, she was diagnosed to be a case of type III Klippel Feil syndrome (KFS) with crossed fused renal ectopia (left to right) and pelviureteric junction obstruction (PUJO) of the right moiety. The patient underwent successful pyeloplasty of the right moeity. To the best of our knowledge, this is the first reported case in the literature with a combination of KFS, crossed fused renal ectopia, and PUJO.

Estimating under-reporting of COVID-19 cases in Indian states: an approach using a delay-adjusted case fatality ratio.

OBJECTIVES: The COVID-19 pandemic has spread to all states in India. Due to limitations in testing coverage, the true extent of the spread may not be fully reflected in the reported cases. In this study, we obtain time-varying estimates of the fraction of COVID-19 infections reported in the different states. METHODS: Following a methodology developed in prior work, we use a delay-adjusted case fatality ratio to estimate the true fraction of cases reported in different states. We also develop a delay adjusted test positivity estimation method and study the relationship between the estimated test positivity rate for each state and the estimated fraction of cases reported. SETTING: We apply this method of analysis to all Indian states reporting at least 100 deaths as of 10 October 2020. RESULTS: Our analysis suggests that delay-adjusted case fatality ratios observed in different states range from 0.47% to 3.55%. The estimated fraction of cases reported in different states ranges from 39% to 100% for an assumed baseline case fatality ratio of 1.38%, from 18.6% to 100% for an assumed baseline case fatality ratio of 0.66%, and from 2.8% to 19.7% for an assumed baseline case fatality ratio of 0.1%. We also demonstrate a statistically significant negative relationship between the fraction of cases reported in each state and the testing positivity rate. CONCLUSIONS: The estimates provide a means to quantify and compare the trends of reporting and the true level of current infections in different states. This information may be used to guide policies for prioritising testing in different states, and also to analyse the time-varying effects of different quarantine measures adopted in different states.

A New Link to Primate Heart Development.

Long non-coding RNAs (lncRNAs) are important regulators of development. In this issue of Developmental Cell, Wilson et al. utilize pluripotent stem cell models to demonstrate that a primate lncRNA, BANCR, is primarily expressed in fetal cardiomyocytes and promotes cell migration.

Methods for Endoscope Reprocessing.

Over the past 2 decades, in hospital centers worldwide, there have been numerous outbreaks of multidrug-resistant organisms that have since been attributed to endoscopic transmission of the infections between patients, primarily from duodenoscopes. These outbreaks have focused the attention of endoscope manufacturers, professional societies, and regulatory agencies on improving the reprocessing of these devices. The key steps in this process are point-of-use precleaning, leak testing, manual cleaning, high-level disinfection, and finally drying and storage. The promise of these initial efforts suggest that the aim of minimizing and ultimately eliminating events of endoscope-/duodenoscope-associated transmission of infectious organisms between patients can be achieved.

N6-methyladenine in DNA antagonizes SATB1 in early development.

The recent discovery of N6-methyladenine (N6-mA) in mammalian genomes suggests that it may serve as an epigenetic regulatory mechanism1. However, the biological role of N6-mA and the molecular pathways that exert its function remain unclear. Here we show that N6-mA has a key role in changing the epigenetic landscape during cell fate transitions in early development. We found that N6-mA is upregulated during the development of mouse trophoblast stem cells, specifically at regions of stress-induced DNA double helix destabilization (SIDD)2-4. Regions of SIDD are conducive to topological stress-induced unpairing of the double helix and have critical roles in organizing large-scale chromatin structures3,5,6. We show that the presence of N6-mA reduces the in vitro interactions by more than 500-fold between SIDD and SATB1, a crucial chromatin organizer that interacts with SIDD regions. Deposition of N6-mA also antagonizes SATB1 function in vivo by preventing its binding to chromatin. Concordantly, N6-mA functions at the boundaries between euchromatin and heterochromatin to restrict the spread of euchromatin. Repression of SIDD-SATB1 interactions mediated by N6-mA is essential for gene regulation during trophoblast development in cell culture models and in vivo. Overall, our findings demonstrate an unexpected molecular mechanism for N6-mA function via SATB1, and reveal connections between DNA modification, DNA secondary structures and large chromatin domains in early embryonic development.