Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Ingrowing Liver as Atypical Recurrent Diaphragmatic Hernia Presentation&mdash;Diagnostic and Treatment Difficulties: A Case Report

(1) Introduction: Recurrent diaphragmatic hernia is a relevant diagnostic and treatment dilemma. We have presented a patient with ingrowing liver as an atypical diaphragmatic hernia recurrence and discussed major aspects of diagnostic methods and the selection of an appropriate operative treatment. (2) Case description: We discuss a case of a patient with right-sided recurrent CDH (Congenital Diaphragmatic Hernia) who had primary thoracoscopic repair in newborn period. During infancy and early childhood, the patient presented recurrent upper and lower respiratory tract infections and bronchial hyperreactivity. The clinical picture was initially unclear. A CT scan was inconclusive to diagnose a recurrence. The patient was scheduled to have a re-thoracoscopy. A part of the liver was herniated into the pleural cavity. This fragment of &lsquo;ingrowing&rsquo; liver was removed, and the diaphragmatic secondary defect was repaired. (3) Conclusions: This case proved that thoracoscopy can be a preferred technique in the diagnosis and treatment of CDH recurrence

Reliability and Validity of the Polish Version of the Esophageal-Atresia-Quality-of-Life Questionnaires to Assess Condition-Specific Quality of Life in Children and Adolescents Born with Esophageal Atresia

Aim: This study reports the reliability and validity of the Polish version of the Esophageal Atresia Quality of Life (EA-QOL) questionnaires, which were originally developed in Sweden and Germany. Methods: A total of 50 families of children (23 aged 2 to 7, and 27 aged 8 to 17) with EA/TEF (esophageal atresia/tracheoesophageal fistula) participated in the study. The development and validation of the Polish version of the EA-QOL involved forward-backward translation of the survey items following the guidelines for cross-cultural translation, cognitive debriefing and evaluation of psychometric properties, including assessment of internal and retest reliability, linguistic validity, content validity, known-group validity and convergent validity. The medical records of patients and standardized questionnaires were used to obtain clinical data. The level of significance was p &lt; 0.05. Results: The Polish versions of the EA-QOL questionnaires demonstrated strong linguistic and content validity, are slightly discriminative for esophageal and respiratory problems, but do not show convergent validity with the PedsQL 4.0 generic core scales. In terms of reliability, the internal consistency of the subscale and total scale of Polish versions as measured by Cronbach&rsquo;s alpha is good, and retest reliability is excellent. Conclusions: The Polish versions of the EA-QOL questionnaires meet most psychometric criteria that confirm the EA-QOL questionnaires&rsquo; reliability and validity. This study enables application of these questionnaires in future research among children with EA in Poland and participation in international multicenter studies focusing on advancing knowledge of condition-specific QOL in this population. Future cross-cultural research using larger sample sizes is still needed to better address the relationship between condition-specific and generic QOL, as well as the discriminative ability of the EA-QOL questionnaires

Laparoscopic ureteroneocystostomy in the treatment of urinary incontinence due to ectopy of the ureters in female dogs: A pilot study.

Ureteral ectopia is rare and requires surgical treatment after a thorough diagnostic workup. Open surgical techniques for repositioning ectopic ureters have been known for many years and are well described in the literature. However, to the best of our knowledge, no laparoscopic method of correcting this pathology has been described, which, in our opinion, would benefit the animal in terms of the healing process and overall clinical outcomes. This study aimed to evaluate the possibility of laparoscopic treatment of ureteral ectopia, which causes urinary incontinence in dogs. All of the operated ten dogs presented in this study were client-owned females with symptoms of urinary incontinence due to a unilateral intramural ectopic ureter. A three-trocar laparoscopic technique was used to perform the ureteroneocystostomy of the ectopic ureter. In this article, clinicopathological data, imaging features, procedural findings, complications, and short- and long-term outcomes are presented. The procedure was feasible in all cases. No major postoperative complications were observed. Among the minor complications, slight hematuria was observed in three dogs, which resolved spontaneously. In the period of at least one year after surgery, no negative impact of the procedure was observed. Seven of the ten operated dogs regained urinary continence. The remaining three dogs required additional surgery (urethral bulking) because of a lack of improvement after adjuvant pharmacological treatment. Overall, good-to-excellent long-term outcomes can be achieved; however, dogs that remain incontinent after laparoscopic ureteroneocystostomy may require additional treatment

Epigenetic Findings in Twins with Esophageal Atresia

Esophageal atresia (EA) is the most common malformation of the upper gastrointestinal tract. The estimated incidence of EA is 1 in 3500 births. EA is more frequently observed in boys and in twins. The exact cause of isolated EA remains unknown; a multifactorial etiology, including epigenetic gene expression modifications, is considered. The study included six pairs of twins (three pairs of monozygotic twins and three pairs of dizygotic twins) in which one child was born with EA as an isolated defect, while the other twin was healthy. DNA samples were obtained from the blood and esophageal tissue of the child with EA as well as from the blood of the healthy twin. The reduced representation bisulfite sequencing (RRBS) technique was employed for a whole-genome methylation analysis. The analyses focused on comparing the CpG island methylation profiles between patients with EA and their healthy siblings. Hypermethylation in the promoters of 219 genes and hypomethylation in the promoters of 78 genes were observed. A pathway enrichment analysis revealed the statistically significant differences in methylation profile of 10 hypermethylated genes in the Rho GTPase pathway, previously undescribed in the field of EA (ARHGAP36, ARHGAP4, ARHGAP6, ARHGEF6, ARHGEF9, FGD1, GDI1, MCF2, OCRL, and STARD8)

Fig 1 -

Cystoscopic assessment, patient positioning for surgery and stages of ectopic ureter preparation for laparoscopic ureteroneocystostomy: A: Cystoscopic view of an ectopic ureter (lower left corner) opening into the distal urethra (UR). B—Patient positioning for the laparoscopic ureteroneocystostomy in lateral recumbency with the affected (ectopic) side up;—the blue arrow points to the cranial part of the operated animal. C: Trocar placement during laparoscopic ureteroneocystostomy. Working trocars, blue arrows; optic trocar, red arrow. D: Laparoscopic intraoperative view of the ectopic ureter (EU) before dissection from the surrounding tissues. B, urinary bladder; UT, uterus; R, rectum. E, F: Laparoscopic operative view of the subsequent stages of ectopic ureter (EU) preparation from the surrounding tissues. BL: Bladder lateral ligament. During preparation, the grasp has to be of the tissues surrounding the ureter, not the ureter itself, to avoid damage. The blue arrow shows the correct way to hold the ureter when preparing.</p

Laparoscopic intraoperative view of the anastomosis of the ureter with the bladder wall and the obtained postoperative result.

A: Placing a suture between the ureter and bladder wall. Ectopic ureter (EU) after transposition into a new opening in the bladder (B). The blue arrow indicates the needle used during suturing. B—View of the performed anastomosis: blue arrows indicate the suture site. C: View of the postoperative wounds after the working trocars (blue arrows) and the optic trocar (red arrow); the yellow arrow indicates the cranial part of the patient. D—View of the transpositioned ureter (blue arrow) two months after surgery during control cystoscopy in a female dog with persistent urinary incontinence.</p

Fig 2 -

Laparoscopic intraoperative view of different stages of the ectopic ureter transposition: A—Ligation of the ectopic ureter (EU) and sliding knot (blue arrow); B, the urinary bladder. B—Red arrow indicates vessel coagulation on the surface of the ectopic ureter (EU) at the level of the planned transverse ureter incision. The blue arrow indicates the site of the previous ligation. B—urinary bladder. C: Transverse cut of the ureter (red arrow) near the previously placed ligation (blue arrow). EU: ectopic ureter. B: urinary bladder. D: Ureter is cut parallel to its long axis before transposition into the bladder lumen (red arrow); EU: ectopic ureter; B: urinary bladder. E—Placing the dissected and cut-off ectopic ureter (EU) to determine a spot for transposition to the bladder (B), which reduces the risk of excessive tension at the place of subsequent anastomosis. The red arrow indicates the laparoscopic hook and designated ureteral transposition site. F: Orifice in the bladder (B) wall of the transposed ureter (B). The diameter of the orifice was approximately equal to the outer diameter of the ureter. The red arrow indicates the bladder lumen after mucosal incision.</p