Type Of Atresia Research Articles

Assessment of the Concerns of Caregivers of Children with Repaired Esophageal Atresia-Tracheoesophageal Fistula Related to Feeding-Swallowing Difficulties.

The study aimed to assess concerns of caregivers of children with EA-TEF related to feeding-swallowing difficulties, compare the concerns according to type of atresia and repair time, and investigate its relationship with time to start oral feeding. Caregivers accompanying 64 children with EA-TEF were included. Age, sex, type of atresia, repair time, and time to start oral feeding were noted. Parents completed the Turkish version of the Feeding/Swallowing Impact Survey (T-FS-IS) to assess the concerns of caregivers related to feeding-swallowing difficulties. The T-FS-IS has three subscales including daily activities, worry, and feeding difficulties. The median age of patients was 3 (min = 1, max = 12) years, of which 57.8% were male. 43.8% of cases were isolated-EA, and 56.3% were EA-distal TEF. 57.8% of cases received early repair, and 42.2% had delayed repair. The median time to start oral feeding was 4weeks (min = 1, max = 128). The mean scores of daily activities, worry, feeding difficulties, and total score from the T-FS-IS were 2.43 ± 1.18, 2.73 ± 1.28, 2.10 ± 0.97, and 2.44 ± 1.09, respectively. Caregivers of children with isolated-EA reported more problems in total score and all subscales of the T-FS-IS than EA-distal TEF (p < 0.01). Caregivers of children who received delayed repair reported more problems in total score and all subscales of the T-FS-IS than children with early repair (p < 0.05). Moderate to strong correlations were found between the T-FS-IS and time to start oral feeding (p < 0.01, r = 0.55-0.65). This study suggests that caregivers of children with isolated-EA and/or delayed repair and/or delay in oral intake may have higher concerns related to feeding-swallowing difficulties.

Therapeutic strategy for thoracoscopic repair of esophageal atresia and its outcome.

Thoracoscopic repair can be safely performed in most types of congenital esophageal atresia (EA), including in patients with long gap EA or very low birth weight. Accordingly, we performed single- or multistage thoracoscopic repair for various EA types. We aimed to report our therapeutic strategy for thoracoscopic radical surgery for treating EA and its outcome. Outcomes of radical surgeries for treating congenital EA at our institute from 2013 to 2018 were retrospectively evaluated. Thirty-eight radical surgeries were evaluated: 3Gross type-A, 1type-B, 30type-C, 1type-D, and 3type-E. The cervical approach was performed in 5cases and thoracoscopic esophageal anastomosis in 33,including 26single-stage (all type-C) and 7multistage surgeries (3type-A, 3type-C, and 1type-D). There were no cases of thoracotomies or intraoperative thoracoscopic surgery complications. Three cases of minor leakage were conservatively resolved. Three postoperative chylothorax surgeries (9%) and seven balloon dilatations (21%) for anastomotic stenosis were performed. Thoracoscopic radical surgery for treating EA, including single- and multistage procedures, can be performed, except in type-E cases or when the end of the proximal esophagus is located higher than the clavicle.

Outcomes of end-to-side oblique anastomosis as a surgical technique for jejuno-ileal atresia.

All of the anastomotic techniques used for jejuno-ileal atresia aim to overcome the size discrepancy between the resected bowel ends, lessen anastomotic complication and prevent development of short bowel syndrome. Aim of study was to evaluate the outcomes of end-to-side oblique anastomosis for jejuno-ileal atresia and verifying the risk factors for complications. A prospective study was conducted on patients with jejuno-ileal atresia managed by end-to-side oblique anastomotic technique over a 7-year period. Data were collected and analyzed to evaluate the outcomes of this surgical technique as morbidity and mortality. The study included 40 patients, 26 males and 14 females. The mean operation time was 105 minutes, the mean time for starting oral feeding was 5.7 days and the mean duration of hospital stay was 9.13 day. Postoperative complications occurred in 11 patients (27.5%). Death was recorded in six cases; survival rate was 85%. End-to-side oblique anastomosis is simple and effective surgical procedure for most types of jejuno-ileal atresia. The technique results in wide and early functioning anastomosis, relatively low morbidity and mortality. As with any neonatal surgery, the overall prognosis and outcomes is primarily dependent on prematurity, birth weight and associated anomalies in addition to the amount of residual bowel function that exist after surgery.

The relationship between oxidative stress markers in exhaled breath condensate and respiratory problems in patients with repaired esophageal atresia

AimTo evaluate the relationship between respiratory problems and oxidative stress markers in exhaled breath condensate (EBC) of patients with esophageal atresia (EA). MethodsEA cases with respiratory problems were evaluated retrospectively for age, gender, the type of atresia, surgical treatment, outcome and respiratory symptoms. The results of gastroesophageal reflux (GER) treatment including the use of proton pump inhibitor (PPI) and fundoplication were also documented. EBC samples of 500–1000 μl were obtained by Ecoscreen machine in all cases. The levels of Glutathione (Glut), 8-isoprostane (8-iso), cysteinyl-leukotriene (Cys-LT) were measured with ELISA. Results were compared with healthy control subjects (CG, n = 26) and the relationship between oxidative stress markers and respiratory symptoms was evaluated. The results of GER treatment and oxidative stress markers in EBC were also correlated. ResultsTwenty-nine patients with a mean age of 8.8 years (3–14 years) were included. The male/female ratio was 16:13. The EA presented with distal fistula in 27 cases. While no fistula was observed in 1 case, both proximal and distal fistulae were present in another single case. Associated anomalies, most of which were cardiovascular anomalies, were observed in 65.5% (n = 19) of cases. The median Glut level was 1.03 mM/ml (0.93–1.15), iso-8 was 38.8 pg/mL (32.03–76.2) and Cys-LT was 0.44 pg/mL (20.17–61.3) in patients with EA. The median levels of oxidative markers in CG were 1.23 mM/mL (1.13–1.36), 66.3 pg/mL (33.5–106.7), and 56.9 pg/mL (27.4–80.1), respectively. Glut levels were significantly lower in EA cases compared to CG (p = 0.01). There was no significant difference between the groups regarding 8-iso and CYS-LT levels (p = 0.9, p = 1.0). Cys-LT levels were significantly lower in patients with PPI treatment [21.7 pg/mL (18.6–48.1)], when compared to patients without PPI treatment [41.1 pg/mL (22.5–83.1)] (p = 0.04) and healthy subjects [56.9 pg/mL (27.4–80.1)] (p = 0.017). The 8-iso levels were significantly decreased in cases with fundoplication compared to the patients without fundoplication (p = 0.02). ConclusionGlut – an antioxidant agent – levels were significantly lower in EBC of EA cases. The decrease in Cys-LT levels in cases with PPI treatment and in 8-iso levels in patients with fundoplication suggests that the oxidative damage in EBC of EA cases may be correlated with GER and its management. Type of studyCase control study Level of evidenceLevel III

DOZ047.12: A comparative analysis of concerns of caregivers with repaired esophageal Atresia-Tracheoesophageal fistula

Abstract Aim Children with esophageal atresia and tracheoesophageal fistula (EA-TEF) may experience feeding and swallowing difficulties, which result in stressful interactions between children and caregivers, and potentially impact the concerns of caregivers. The aim of this study was to assess concerns of caregivers of children with EA-TEF related to feeding–swallowing difficulties and compare the concerns according to type of atresia, repair time and time to start oral feeding. Methods Caregivers accompanying 24 children with EA-TEF were included. Age, sex, type of atresia, repair time, and time to start oral feeding of children were noted. Parents completed the Feeding/Swallowing Impact Survey (FS-IS) to assess the concerns of caregivers related to feeding–swallowing difficulties. It has three subscales, including daily activities, worry, and feeding difficulties. Average scores range between 1 and 5, of which increasing scores reflect more caregiver concern. Results The mean age was 3.41 ± 2.71 years, of which 66.7% were male. 56.3% of cases were isolated EA, and 43.8% were EA–distal TEF. 60.9% of cases received early repair (&lt;1 month of age), and 39.1% had delayed repair (between 1 and 12 months). The median time to start oral feeding was 4 weeks (min = 2, max = 128). The mean scores of daily activities, worry, feeding difficulties, and the total score were 2.40 ± 1.01, 2.79 ± 1.06, 2.14 ± 0.95, and 2.49 ± 0.88, respectively. Caregivers of children with isolated EA reported more problems with ‘daily activities’, ‘feeding difficulties’, and ‘total score’ than EA–distal TEF (P &lt; 0.05). Caregivers of children who received delayed repair reported more problems with the ‘daily activities’ subscale and ‘total score’ than children with early repair (P &lt; 0.05). Moderate to strong correlations were found between the ‘daily activities’ and ‘feeding difficulties’ subscales of FS-IS and time to start oral feeding (P &lt; 0.05, r = 0.56–0.69). Conclusions This study suggests that concerns of caregivers of children with EA-TEF related to feeding–swallowing difficulties are associated with the surgical outcome of EA. Caregivers of children with isolated-EA and/or delayed repair and/or delay in oral intake may have higher concerns related to feeding–swallowing difficulties.

DOZ047.05: The flip side of the coin

Abstract Background Being two medical doctors (family practitioner and pediatric surgeon) with a son born with a rare type of esophageal atresia we felt the need to explore the nonmedical side of this disease. Aim The aim of this study was to emphasize the role of emotional support for parents with children born with all types of esophageal atresia and to realize the importance of the nonsurgical side—the flip side of the coin, to explore the need for support not only for the patient but also the family. Literature Review and Personal Experience The epidemiology, surgical management, and biological outcome of esophageal atresia with/without trachea-esophageal fistula are well known. The softer, more humane side of this condition, the flipside of the coin, being the cause of PTSD children with OA (mothers &gt; fathers).2 Severe anxiety also seems to be present in some of these parents. A meta-analysis done in 2013 concluded that psychological morbidity associated with esophageal atresia has important implications for clinical practise3; namely, if psychological support is being offered to parents during the neonatal period and continues as part of the long-term follow-up for both parents and the patients, the overall outcome is improved and the biopsychosocial morbidity of the parents decreases. Personal experience has taught us that a good support system is a critical part of a child's clinical treatment plan. Parents need good emotional support in order to be there for their child when he or she needs them. Conclusion Surgeons look at diseases from a technical point of view. They ask how they can surgically fix the problem and improve the patient's quality of life. They often forget the other side that parents need emotional support, someone to be there for them and to listen to their experience in hospital and at home. The family as another patient needs to be treated and included in the overall treatment plan if the intention is to improve the quality of life for both the patients and their families. There is a need to develop a multidisciplinary support system in South Africa to aid patients born with OA and their caring families. This includes the entire family and is not limited to the mothers but also includes fathers and other siblings often overlooked.

DOZ047.69: Tracking outcomes of tracheoesophageal fistula and esophageal atresia in pediatric population

Abstract Purpose The purpose of this study is to conduct a retrospective chart review of patients with a rare congenital deformity, tracheoesophageal fistula, and esophageal atresia treated at a small, freestanding Children's Hospital. The goal is to identify important variables to inform clinical pathways and prospective database creation. Current national surgical quality databases do not track tracheoesophageal fistula/esophageal atresia (TEF/EA)-specific data points such as TEF-type and incidence of postoperative complications such as gastroesophageal reflux disease (GERD) and esophageal stricture. Methods Patients diagnosed with esophageal atresia and trachesophageal fistula between January 2005 and January 2019 were identified using the appropriate ICD 9 or ICD 10 codes. Data for 35 variables including patient demographics and type of esophageal atresia were abstracted. SPSS 16.0 software was used for descriptive statistics. Results A total of 43 patients were identified, 64.3% female and 35.7% male. 77.5% were type C, 12.5% type A, 7.5% type H, and 2.5% type D. 15% were long-gap type. 53.8% had postoperative esophageal strictures, and 66.7% had postoperative gastroesophageal reflux disease. Of those who had strictures, 77.2% of patients required at least three postoperative dilations for stricture, with an overall mean six dilations. 26.2% of patients had tracheomalacia; of these, two patients underwent thoracoscopic aortopexy. Four patients were not included in subgroup analysis: two patients had missing variables, one patient passed away before surgical intervention, and one patient has not yet undergone surgical intervention for EA/TEF. Conclusions We conclude that, although a rare congenital anomaly, a database dedicated to tracheoesophageal fistula and esophageal atresia at a pediatric hospital can aid in reviewing trends in outcomes and inform standardization of management.

DOZ047.29: Pleural flap for partial esophageal substitution in case of difficult anastomosis

Esophageal reconstruction in long-gap atresia remains challenging for the surgeon. Complications of surgery, regardless of choices and methods proposed, are frequents, sometimes serious, and can significantly delay baby's feeding, with increased disruption of orality. The ideal is a reconstruction with only esophageal tissue available. There is renewed interest for technical of elongation described initially by Foker, especially modified by Van Der Zee.1 But, elongation is not always so simple that provided. We report an observation of esophageal atresia (type 1 Ladd), treated by rapid elongation. On the sixth day of procedure, right pleurisy reveals a rip at the apex of inferior esophagus. Anastomosis was possible on the posterior plane, but impossible on the anterior plane. The anterior plane has been reconstituted with a flap of parietal pleura. The operative course was simple without any fistula. Oral feeding started at fifteenth day postanastomosis was quickly complete. Gastrostomy left by security has not been used. Three endoscopic dilations have been necessary at six weeks, two and five months after anastomosis. At the age of eleven months, the orality was perfect, and diet was diversified with pieces. Use a pleural flap to bury or isolate sutures is well known technic.2 But use only pleura to replace the esophageal wall is not described. Partial reconstruction of a hemiesophageal wall with only pleura is possible, despite difficult conditions. It is a trick easily achievable in case of difficulty, with a good result that is maintained over time.

DOZ047.105: Minimally invasive surgical approach for posterior tracheobronchopexy to treat severe tracheobronchomalacia

Abstract Purpose Posterior tracheobronchopexy directly addresses membranous airway intrusion in severe tracheobronchomalacia (TBM). Our experience of posterior tracheobronchopexy through an open approach in a large series of patients has been previously reported by us. This study aimed to review lessons learned from our initial series of posterior tracheobronchopexy through a minimally invasive surgical approach. Methods A retrospective review of all patients with symptomatic TBM who underwent video-assisted or robot video-assisted thoracoscopic posterior tracheobronchopexy between October 2016 and January 2019 was carried out. Results Fourteen patients underwent video-assisted (n = 4) or robotic video-assisted (n = 10) thoracoscopic posterior tracheobronchopexy (age range: 8 months–19 years). Two patients had a history of esophageal atresia (type C) repair; none of the other patients had undergone prior operations. Two patients required open conversion to achieve the desired precision of suture placement. Operative times ranged from 4.5 to 15 hours, depending on the additional procedures performed. The intraoperative bronchoscopic evaluation demonstrated marked improvement in airway patency of all patients at the conclusion of each case. Patients were hospitalized 2 to 7 days, with 0 to 4 days for ICU observation. One patient with bronchopulmonary dysplasia and diffuse TBM, including segmental bronchi, required longer hospitalization and prolonged mechanical ventilation support. Follow-up after surgery ranged from 1 to 27 months. Three patients required subsequent anterior aortopexy and tracheopexy. In these three cases, dynamic tracheobronchoscopy showed that posterior tracheopexy remained intact; nonetheless, anterior airway support was still required to prevent dynamic anterior airway collapse. Conclusions The thoracoscopic approach for posterior tracheobronchopexy, while challenging, can be applied in children with severe TBM. As experience is gained, more complicated procedures and reoperative cases are possible. The simultaneous use of flexible bronchoscopy is mandatory to confirm precision by providing luminal visualization during suture placement. Thoracoscopic surgery with robotic assistance can eliminate some technical limitations of the video-assisted approach by providing an easier platform for very complicated suturing angles.

DOZ047.78: Endoscopic management of symptomatic tracheal pouches with trichloroacetic acid chemocauterization

Abstract Introduction Trichloroacetic acid (TCA) has been used in different conditions such as recurrent and congenital tracheoesophageal fistula and branchial sinus of the pyriform fossa. Nevertheless, it has never been described as a treatment for symptomatic tracheal pouches after an esophageal atresia repair. This study reports two cases of endoscopic treatment with TCA of symptomatic residual tracheal pouches in 2 children with repaired type III esophageal atresia. Case Reports Two cases of 7 and 8 years with esophageal atresia type III repaired in the neonatal period. Both were diagnosed with severe tracheomalacia and presented recurrent pulmonary infections complicated with pulmonary bronchiectasis. One of the patients benefited from a Nissen fundoplication due to severe gastroesophageal reflux at 2 years of life. Due to persistent respiratory infections bronchoscopy was indicated detecting a blind 2 cm tracheal pouch at the level of the carina in both patients. Endoscopic treatment by the use of TCA was performed. By rigid bronchoscopy chemo-cauterization of the pouch with 50% TCA followed by a brush abrasion of the mucosa was done. Postoperatively one of the patients developed a slight and limited subcutaneous and mediastinal emphysema due to postoperative cough. No other complications were reported. Endoscopic control at 3 months (and also 12 in 1 case) after procedure showed complete obliteration of the tracheal pouches. After a medium follow-up of 17.5 months (14 and 21) a significant decrease in the number of respiratory infections and hospital admissions has been observed in both cases. Conclusion Endoscopic treatment by the use of 50% TCA in symptomatic tracheal pouches seems safe and effective. The complex surgical corrections of these conditions make it a perfect indication, representing a minimally invasive alternative.

MRI features and differential diagnoses of congenital vaginal atresia

Objective: To investigate the MRI manifestations of congenital vaginal atresia, analyze its imaging features, and improve the understanding of the disease.Methods: MRI findings and clinical data of 12 patients with congenital vaginal atresia confirmed by hysteroscopy and laparoscopic surgery were retrospectively analyzed. Vaginal atresia was classified according to vaginal dysplasia in AFS female genital malformation classification system.Results: In this study, 12 cases of congenital vaginal atresia were diagnosed by combined preoperative MRI with operative diagnosis. Among them, 10 patients all had type-I congenital vaginal atresia, and their uterus and cervix were normal (1 patient had ectopic renal malformation combined with left ovarian endometriosis cyst and 1 patient with uterine empyema). The other two cases were diagnosed congenital vaginal atresia type II (1 case merged with residual uterus, 1 case with cervical dysplasia). MRI mainly manifested as dilatation and hemorrhage in the uterine cavity, cervical canal and vaginal upper segment. T1WI showed high signal, T2WI showed slightly lower and slightly higher signal. The dilated vagina was above the perineal level.Conclusion: MRI features of congenital vaginal atresia have certain characteristics. MRI cannot only accurately assess the type of vaginal dysplasia and its associated complications, but also make objective evaluation and diagnosis, so it can be used as the best effective preoperative image evaluation.

Complications of Corrective Surgery in Preterm Neonates with Congenital Heart Defects: What is the Solution?

Mild congenital heart defects require no treatment, while severe forms of these diseases need immediate interventions. There are several limits in preterm neonates on drug interventions, interventional procedures, and even heart surgery due to the prematurity of pulmonary, renal, and central nervous systems. Considering the mentioned points, the collaboration of other medical professions, particularly neonatologists, is needed in the treatment of these newborns. The application of surgical and interventional instruments are associated with limitations owing to the low birth weight in these neonates. Therefore, it is essential to review and modify the treatment program for this group of patients. By the advancement of surgical treatments mainly in fetal course and the approval of their efficiency, designing a new interdisciplinary field is necessary. In developing countries, such as Iran, the prenatal diagnosis of congenital heart diseases is difficult because of the lack of expertise in this area. On the other hand, abortion seems to be problematic due to the religious reasons. The legal gestational age for abortion is less than 20 weeks, and the probability of dealing with these kinds of diseases is more than developed countries. During several years, we encountered at least 15 cases with the above-mentioned criteria, five of whom required an immediate intervention. Three patients were dextro-Transpotion of the great arteries, one patient had pulmonary atresia with ventricular septal defect, and the two other ones were suffering from coarctation of the aorta and tricuspid atresia type 1A, who all underwent the palliative surgery.

Duodenal Atresia-Tandler’s “Epithelial Plug Stage” Revisited

Background: Duodenal atresia is the most common type of intestinal atresia. Pathogenesis of duodenal atresia can be explained by an embryological theory that involves persistent physiologic epithelial occlusion (“epithelial plug”), first published in 1900. Tandler’s developmental arrest theory has been accepted by the majority of recognized modern textbooks to date. The aim of the presented study is to re-evaluate the relevance of Tandler’s observations. Method: Tandler’s paper was reviewed retrospectively and discussed from the point of view of subsequent research. Results and Discussion: Local epithelial proliferation, vacuolization failure and mesenchyme ingrowth may continuously serve as a partly convincing but incomplete embryologic model to explain membranous duodenal atresia. Tandler’s theory has some weak points regarding the epithelial-mesenchymal interaction, the predisposition of the post-ampullary region, the association of duodenal atresia with other malformations and trisomy 21, and familial occurrence. Shrinkage artifacts, misinterpretation of a three-dimensional problem investigated with a two-dimensional tool (light microscope), animal studies, and the lack of apoptosis call the real existence of a solid stage in early duodenal embryology into question. Conclusion: More sophisticated morphologic, genetic and molecular-biological investigations revealed new insights regarding endoderm to mesoderm signaling as an important key to the pathogenesis of duodenal atresia.

Use of amnion graft and surgicel in vaginoplasty for secondary vaginal atresia: A case report

Vaginal atresia can be congenital or acquired. The tissue is virgin in primary vaginal atresia or agenesis, so various methods have been used with successful outcomes. On the other hand, one can expect a lot of fibrosed tissue in secondary atresia. There is no elaborate literature on secondary vaginal atresia. There is also no standard treatment for both the types of vaginal atresia. The use of amnion graft and surgicel in vaginoplasty for secondary atresia is one of our experiences with best results. We report a case of a 22-year-old primipara who presented with dyspareunia and cyclical lower abdominal pain for the past 4 months. She had a history of traumatic vaginal delivery 3 months prior to presentation. On examination, she was diagnosed with secondary vaginal atresia. We managed her by vaginoplasty with amnion grafting and surgicel, followed by regular dilatation with soft vaginal mould for the next 6 weeks. Our patient is doing well with normal coital function on follow-up.

Effect of Environmental Pollution on Gonads Histology of the Nile Tilapia, Oreochromis niloticus from Lake Manzala, Egypt

Lake Manzala suffers from severe pollution of complex composition from different sources of industrial, agricultural and sewage facilities from the surrounded governorates. The objective of this research was aiming to study the effects of some environmental polluted effluents at five locations of Lake Manzala on the gonads of Oreochromis niloticus. The histological study of testes of O. niloticus showed decreased number of seminiferous tubules with some degenerative changes. They appeared empty from different spermatogenic cells (primary and secondary spermatocytes). Moreover the interstitial cells were collapsed and appeared in all seminiferous tubules. The ovaries presents a deformation from their ideal shapes, thickening and detaching of the membrane of all stages of oocytes and two types of non-bursting atresia in oocytes were markedly appeared. We recommended improving water quality control and the environmental monitoring is essential at Lake Manzala.

Very low bilirubin after portoenterostomy improves survival of the native liver in patients with biliary atresia by deferring liver fibrogenesis

Progression of fibrosis and ensuing complications determine the postoperative course of patients operated on for biliary atresia. We evaluated predictors of the progression of fibrosis in the native liver after operative treatment. Among patients whose bilirubin decreased to <34 µmol/L after portoenterostomy (n = 41), predictors of follow-up cirrhosis and the progression of fibrosis were analyzed with logistic regression and survival of their native liver was evaluated with the Kaplan-Meier method. Areas under receiving operating characteristic were used to define optimal cutoffs. After median follow-up of 5.2 years (interquartile range 1.6-10.2) after portoenterostomy, liver biopsies showed cirrhosis in 53% of patients, and the Metavir stage remained stable or decreased in 38%. The development of cirrhosis was predicted by total or conjugated bilirubin ≥170/120 µmol/L at the time of portoenterostomy (P ≤ .009); normalization of bilirubin within 1.9 months (P = .002); total or conjugated bilirubin ≥ 12.5/7.5 µmol/L (P = .002) and aspartate aminotransferase-to-platelet ratio ≥ 0.55 at 3 months postoperatively (P = .001); and total or conjugated bilirubin ≥ 7.5/2.5 µmol/L (P ≤ .001), aspartate aminotransferase-to-platelet ratio ≥ 0.63 (P = .004), and gamma glutamyl transferase ≥ 266 U/L (P = .007) at 6 months postoperatively. In multiple regression analysis, conjugated bilirubin ≥ 2.5 µmol/L at 6 months increased the risk of cirrhosis 35-fold (P = .020), and other predictors were not predictive. Total or conjugated bilirubin < 12.5/7.5 µmol/L (P ≤ .014), aspartate aminotransferase-to-platelet ratio < 0.55 at 3 months (P = .006), and total or conjugated bilirubin < 7.5/2.5 µmol/L at 6 months postoperatively (P ≤ .014) were the strongest predictors of a stable, nonprogressive fibrosis. Decreases in total or conjugated bilirubin to < 12.5/7.5 µmol/L by 3 months and to < 7.5/2.5 µmol/L by 6 months improved survival of the native liver (log-rank P ≤ .025). Age at follow-up or at portoenterostomy, anatomic type of biliary atresia, use of postoperative steroids, and episodes of cholangitis were unrelated to the progression of fibrosis or the development of cirrhosis (P = not significant). Among patients whose serum bilirubin normalizes after portoenterostomy, its rapid decrease to very low levels prolongs the survival of their native liver by delaying the progression of fibrosis.

Fish consumption in early pregnancy and congenital gastrointestinal tract atresia in the Japan Environment and Children’s Study

Current evidence suggests that the aetiology of congenital gastrointestinal (GI) tract atresia is multifactorial, and not based solely on genetic factors. However, there are no established modifiable risk factors for congenital GI tract atresia. We used data from a Japanese nationwide birth cohort study launched in 2011, and examined whether fish consumption in early pregnancy was associated with congenital GI tract atresia. We analysed data of 89 495 women (mean age at delivery=31·2 years) who delivered singleton live births without chromosomal anomalies. Based on the results of the FFQ, we estimated the daily intake of fish and n-3 PUFA consumption in early pregnancy. We defined a composite outcome (oesophageal atresia, duodenal atresia, jejunoileal atresia and/or anorectal malformation) as congenital GI tract atresia. In this population, median fish intake was 31·9 g/d, and seventy-four cases of congenital GI tract atresia were identified. Fish consumption in early pregnancy was inversely associated with the composite outcome (multivariable-adjusted OR for the high v. low consumption category=0·5, 95 % CI 0·3, 1·0). For all the specific types of atresia, decreased OR were observed in the high consumption category, although not statistically significant. Reduced atresia occurrence was observed even beyond the US Food and Drug Administration's recommended consumption of no more than 340 g/week. Also, n-3 PUFA-rich fish and n-3 PUFA consumptions tended to be inversely associated with atresia. Fish consumption in early pregnancy may be a preventive factor for congenital GI tract atresia.

Prognostic Factors and Management of Patients with Choanal Atresia

To analyze prognostic factors in the management of patients with choanal atresia. This is a review of 114 patients operated on for choanal atresia in a tertiary care center between November 1986 and November 2016, including clinical characteristics, surgical management, and postoperative course with final nasal patency. The data were collected in a database that was updated over time. Among the 114 patients, 78 were female, 77 presented with unilateral choanal atresia, and 37 presented with bilateral unilateral choanal atresia, corresponding to 151 nasal fossae. Forty-seven patients had associated abnormalities (41.2%), including CHARGE, diagnosed in 20 children (17.5%). At the end of follow-up, 91 patients (79.8%) had normal choanal patency. The identified risk factors of surgical failure were age <6 months (P = .004), weight <5 kg (P = .007), and bilateral choanal atresia (P < .001). The type of atresia, presence of associated abnormalities, surgical approach, stenting, and use of mitomycin were not significantly linked with the surgical results. This series highlights the importance of the timing of surgery, which is guided by the clinical ability of the infant to tolerate the procedure. Endoscopic approaches are widely performed, but a transpalatal approach, necessary in some cases of bilateral choanal atresia, does not alter the results. Unilateral choanal atresia surgery should be delayed after age 6 months and/or weight >5 kg when possible.

Management and outcome of neonates with a prenatal diagnosis of esophageal atresia type A: A population-based study.

Evaluate the neonatal management and outcomes of neonates with prenatal diagnosis of esophageal atresia (EA) type A. This population-based study was conducted using data from the French National Register for infants with EA born from 2008 to 2014, including all cases of EA type A. We compared prenatal and neonatal characteristics and outcomes in children with prenatal diagnosis of EA type A with those with a postnatal diagnosis until the age of 1. A total of 1118 live births with EA were recorded among which 88 (7.9%) were EA type A. Prenatal diagnoses were performed in 75 cases (85.2%), and counselling with a prenatal specialist was conducted in 84.8% of the prenatal group. Still within that group, the gestational age at delivery was significantly higher than in the postnatal group (36 [35-38] versus 34 [32-36] weeks; P=.048). Inborn births were more frequent in the prenatal group (86.1% vs 7.7%, P<.0001), and mortality and outcome were similar in both groups. Prenatal diagnosis is high in EA type A, which enables to offer an antenatal parental counseling and which avoids postnatal transfers. Prognosis of EA types A does not appear to be influenced by the prenatal diagnosis.

Obliterative cholangiopathy in acquired cystic biliary atresia type III after cyst perforation: a case report

BackgroundIn biliary atresia, the disease process of obliterative cholangiopathy may begin in the perinatal period; however, no chronological evidence exists on how the cholangiopathy progresses to biliary obliteration.This is the first acquired case with the final diagnosis of type III cystic biliary atresia with an extrahepatic biliary cyst which showed the progression of obliterative cholangiopathy in chronological order after birth.Case presentationAn 81-day-old girl presented with acute abdominal distress due to bilious peritonitis caused by biliary cyst perforation, for which she underwent emergency biliary drainage. Postoperative images showed a dilated common bile duct and hepatic ducts bilaterally, with flow of the contrast medium to the duodenum through the dilated common bile duct. Biochemistry of the bile collected during and after the operation revealed elevated levels of pancreatic enzymes in the bile from the gallbladder. The patient was diagnosed as having a congenital choledochal cyst and underwent laparotomy at 120 days of age which revealed that she had pancreaticobiliary maljunction. The biliary cyst was resected at the narrow portion just above the junction with the main pancreatic duct. During dissection up to the hepatic hilum, we found that the hilar hepatic ducts were bilaterally replaced by fibrous tissue and were obstructed, leading to a diagnosis of type III a1, μ biliary atresia. The fibrous tissue was excised, and hepatic portoenterostomy was performed according to the Kasai procedure. The patient’s postoperative course was uneventful and the jaundice resolved within 1 month. She has had normal liver function tests with no episode of cholangitis for 3 years after discharge.ConclusionsWe demonstrated the process of acquired type III biliary atresia in a patient with cystic biliary atresia and biliary cyst perforation. To the best of our knowledge, this is the first case of acquired cystic biliary atresia showing chronological progression of the course of obliterative cholangiopathy, providing a better understanding of the development of type III biliary atresia as an acquired disease.