Primary Liver Transplant in Biliary Atresia: The Case for and Against.

The role of liver transplantation as a primary procedure in biliary atresia has been argued over for at least 40 years, indeed since the coming of age of safe liver transplantation during the 1980s. Yet, it is not a common option in most series (usually ≤5%) and typically reserved for those with late presentations (arguably >100 days) with established cirrhosis. This review presents the pros and cons of primary liver transplant. The pros are based upon the observation that at best a Kasai portoenterostomy (KPE) is simply palliative in most, and at worse has no effect whatsoever on restoration of bile flow and is therefore pointless. Set against this are the cons: there is a dearth of prognostic tests (clinical, biochemical, or histological) at the time of presentation which may predict inevitable failure; the possibility of long-term native liver survival to adulthood in a proportion (albeit a minority); and the implied increased need for donor organs suitable for infants - a stressor for an already overstressed system. Improving results from KPE in terms of increasing the proportions clearing their jaundice and minimizing the effects of chronic liver fibrosis and cirrhosis would surely limit the siren calls for primary transplants but the key must be better discrimination at presentation with the use of biomarkers (circulatory or histological, individually or together) to enable better decision making.

Biliary atresia in Uganda: Current ethical challenges and advancement of public policy.

Biliary atresia is a progressive cholangiopathy in neonates, which often results in liver failure. In high-income countries, initial treatment requires prompt diagnosis followed by Kasai portoenterostomy. For those with a late diagnosis, or those in whom Kasai portoenterostomy fails, liver transplantation is the only lifesaving treatment. Unfortunately, in low- and middle-income countries, timely diagnosis is a challenge and liver transplantation is rarely accessible. Here, we discuss the ethical dilemmas surrounding treatment of babies with biliary atresia in Uganda. Issues that require careful consideration include: risk of catastrophic health expenditure to families, ethical dilemmas of transplant tourism, medical risks of maintaining the transplant in a low-resourced health system, and difficult decisions encountered by the surgeon caring for these patients. Four distinct models of the patient-physician relationship are applied to biliary atresia in Uganda. These models describe differences in patient and physician roles, and patient values and autonomy. Solid organ transplantation is a rapidly evolving segment of healthcare in Uganda and ongoing policy advancements may shift ethical considerations in the future.

Retro-pancreatic pull-through reconstruction of the hypoplastic portal vein using the autologous mesosystemic shunt vessel in adult living donor liver transplantation: a case report.

BACKGROUND: In liver transplant patients with hypoplastic portal vein (PV), when the narrowed segment is extended too deep into the dorsal side of the pancreas, it is difficult and dangerous to reconstruct the interposition graft from the upper part of the pancreas. Herein, we present a case of PV reconstruction with the autologous mesosystemic shunt vessel from the caudal side of the pancreas in a situation where the narrowed PV was deep, and we discuss the technical details. CASE PRESENTATION: A 25-year-old woman presented with cholestatic liver cirrhosis due to biliary atresia after Kasai procedure. Since her jaundice progressed, she was referred to our hospital for liver transplantation. Laboratory tests showed that her total bilirubin was elevated to 7.6 mg/dL. The Model for End-Stage Liver Disease score was 18, and the Child-Pugh score was 9 (Grade B). She underwent living donor liver transplantation (LDLT) using a right hemi-liver graft procured from her 54-year-old mother. The conventional approach from the cephalad side to the superior mesenteric vein (SMV) and splenic vein (SpV) confluence behind the pancreas was extremely difficult in this case because the confluence of SMV and SpV was close to the lower edge of the pancreas. Therefore, we decided to perform PV reconstruction from the caudal side. The main trunk of PV was documented as narrow (5 mm in diameter), for which retro-pancreatic pull-through PV reconstruction was successfully performed using her own mesosystemic shunt vessel. A contrast computed tomography (CT) scan was performed on postoperative day 5 because of an elevation of D-dimer and found a partial thrombus in the left pulmonary artery, as well as in the PV and left renal vein. Thereafter, thrombolytic therapy with low-molecular-weight heparin was started immediately and switched to a direct oral anticoagulant. The follow-up CT taken 3 months after liver transplantation revealed a patent PV without thrombus; therefore, anticoagulant therapy was discontinued. Currently, the patient has been well and active with a patent PV without anticoagulant therapy for 3 years after LDLT. CONCLUSIONS: Retro-pancreatic pull-through reconstruction of the hypoplastic PV is a feasible and effective method when conventional reconstruction is not indicated.

Thoracic Musculoskeletal Deformities Following Surgical Treatment of Esophageal Atresia - Thoracoscopic Versus Open Approach: A Retrospective Two Centers Cohort Study.

INTRODUCTION: Thoracic musculoskeletal deformities are significant complications following open correction of esophageal atresia (EA) during long-term follow-up. We aimed to compare the frequency and severity of thoracic musculoskeletal deformities after open and thoracoscopic repair of EA. We hypothesized that fewer deformities would occur following the less invasive thoracoscopic approach. METHODS: This retrospective study analyzed patients treated at two pediatric surgery departments in Poland between 2005 and 2021. The patient groups differed in surgical approach, operative techniques, indications for multi-staged surgery, and postoperative complications. The study encompassed all types of EA/TEF. The first group comprised 68 patients who underwent thoracoscopic esophageal atresia repair (Wroclaw), while the second group involved 44 patients who underwent open repair (Warsaw). Clinical data were retrospectively reviewed, with results considered significant at p < 0.05. RESULTS: The median age at examination was 6 years in the thoracoscopy group and 5.5 years in the thoracotomy group. In the thoracoscopy group, 53 out of 68 patients (77.9%) and in the thoracotomy group - 35 out of 44 patients (79.5%) were treated in one stage. The incidence of thoracic musculoskeletal deformities was significantly lower in the thoracoscopy group (1.5%) compared to the thoracotomy group (34.1%, p < 0.001). Scoliosis occurred significantly more often after thoracotomy (13.6% vs 1.5%, p = 0.016). There was no rib fusion (0% vs 37.1%, p < 0.001) and no scoliosis of =>20° (0% vs 6.8%, p = 0.058) after thoracoscopy. The coincidence of rib fusion and scoliosis was significant (9.1%, p = 0.022) for the open approach. In the thoracotomy group, multi-staged surgery and more frequent reoperations due to major complications were significantly associated with an increased occurrence of deformities. None of the patients after thoracoscopic multi-stage or complicated EA/TEF repair developed scoliosis. CONCLUSIONS: The frequency and severity of thoracic musculoskeletal deformities were significantly lower after the thoracoscopic approach. Thoracoscopy may be a more advantageous and preferred surgical approach for the EA/TEF treatment, although further randomized, controlled studies are necessary. Post-thoracotomy scoliosis may progress to a severity requiring surgery.

The Electrified Wire Technique in Complex Aortic Interventions: A Case Series.

OBJECTIVES: Electrosurgery has been long used in endovascular procedures, with only case reports in the aortic field. Our aim is to present a case series with the use of an electrified wire to perform catheter-based electrosurgery by applying external current through an electrocautery pen. METHODS: Single-center retrospective case series of all patients undergoing complex aortic surgery from October 2020 to August 2023, in whom the electrified wire technique was used: (1) Perforation of a dissection flap or left subclavian artery (LSA) in situ endograft fenestration-a 0.014" polytetrafluoroethylene (PTFE) insulated guidewire is detached from the insulation with a scalpel at the end and a cautery pen is here attached with a clamp. A curved tip catheter or sheath is positioned against the aortic flap or the endograft (through a left brachial access in this case) and the wire pushed, crossing the flap by activating the electrocautery pen and (2) slicing a dissection flap ("powered cheese-wire technique")-after same preparation as above, the middle section of the 0.014 guidewire is removed from the PTFE and bent into a V-shape. Once in the aorta, the guidewire crosses from the true lumen (TL) to the false lumen (FL) and a through-and-through access is obtained. Sheaths are positioned against the flap from both sides and moved up or down while the electricity is activated, slicing the flap and communicating both lumens. Technical success and technical-related complications were evaluated. RESULTS: Eleven cases concerning aortic dissections and 1 case of aortic atresia were treated. Four patients presented urgently, whereas the rest were planned procedures. Seven cases underwent perforation of a dissection flap, 2 cases underwent the powered cheese-wire technique, in 2 cases for an LSA in situ fenestration, and in 1 case to cross an aortic atresia at the aortic isthmus. The technique was in all cases successfully applied. No complications related to the technique occurred. CONCLUSIONS: The "electrified wire" technique is a feasible and ready-available tool that can be safely used in complex aortic interventions, especially to perforate aortic tissue like dissection flaps or to perform in situ fenestrated repairs by perforation of the endograft fabric. CLINICAL IMPACT: The electrified wire technique described herein is a straightforward technique that uses readily available tools to perform electrosurgery. We present its use in complex aortic procedures. However, it could be envisioned for any vascular procedure that requires crossing of the vessel or even prosthetic material. As we have described in this series, when used along with an adequate properative planning, it can be a safe tool of great utility, as has already been demonstarted in the field of the interventional cardiology.

Inhibition of Notch3/Hey1 ameliorates peribiliary hypoxia by preventing hypertrophic hepatic arteriopathy in biliary atresia progression.

Emerging evidence indicates the presence of vascular abnormalities and ischemia in biliary atresia (BA), although specific mechanisms remain undefined. This study examined both human and experimental BA. Structural and hemodynamic features of hepatic arteries were investigated by Doppler ultrasound, indocyanine green angiography, microscopic histology, and invasive arterial pressure measurement. Opal multiplex immunohistochemistry, western blot, and RT-PCR were applied to assess Notch3 expression and the phenotype of hepatic arterial smooth muscle cells (HASMCs). We established animal models of Notch3 inhibition, overexpression, and knockout to evaluate the differences in overall survival, hepatic artery morphology, peribiliary hypoxia, and HASMC phenotype. Hypertrophic hepatic arteriopathy was evidenced by an increased wall-to-lumen ratio and clinically manifested as hepatic arterial hypertension, decreased hepatic artery perfusion, and formation of hepatic subcapsular vascular plexuses (HSVPs). We observed a correlation between overactivation of Notch3 and phenotypic disruption of HASMCs with the exacerbation of peribiliary hypoxia. Notch3 signaling mediated the phenotype alteration of HASMCs, resulting in arterial wall thickening and impaired oxygen supply in the portal microenvironment. Inhibition of Notch3/Hey1 ameliorates portal hypoxia by restoring the balance of contractile/synthetic HASMCs, thereby preventing hypertrophic arteriopathy in BA.

"Where Did This Come From?": Antibiotic Prophylaxis in Biliary Atresia After Kasai Procedure.

For patients with Biliary atresia, antibiotic prophylaxis after Kasai portoenterostomy is a common practice. Societal guidelines often cite one reference as supportive evidence for this practice. In this paper, we go back to review the quality of this evidence and suggest more research is required to demonstrate the efficacy of antibiotic prophylaxis in this population.

ARCAPA in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.

We report a case of anomalous origin of the right coronary artery from the pulmonary artery in a patient with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. The diagnosis was made during a proposed hybrid approach to stent the native right ventricular outflow tract, and an alternative surgical strategy was created.

Predictive modeling for early detection of biliary atresia in infants with cholestasis: Insights from a machine learning study.

Cholestasis, characterized by the obstruction of bile flow, poses a significant concern in neonates and infants. It can result in jaundice, inadequate weight gain, and liver dysfunction. However, distinguishing between biliary atresia (BA) and non-biliary atresia in these young patients presenting with cholestasis poses a formidable challenge, given the similarity in their clinical manifestations. To this end, our study endeavors to construct a screening model aimed at prognosticating outcomes in cases of BA. Within this study, we introduce a wrapper feature selection model denoted as bWFMVO-SVM-FS, which amalgamates the water flow-based multi-verse optimizer (WFMVO) and support vector machine (SVM) technology. Initially, WFMVO is benchmarked against eleven state-of-the-art algorithms, with its efficiency in searching for optimized feature subsets within the model validated on IEEE CEC 2017 and IEEE CEC 2022 benchmark functions. Subsequently, the developed bWFMVO-SVM-FS model is employed to analyze a cohort of 870 consecutively registered cases of neonates and infants with cholestasis (diagnosed as either BA or non-BA) from Xinhua Hospital and Shanghai Children's Hospital, both affiliated with Shanghai Jiao Tong University. The results underscore the remarkable predictive capacity of the model, achieving an accuracy of 92.639 % and specificity of 88.865 %. Gamma-glutamyl transferase, triangular cord sign, weight, abnormal gallbladder, and stool color emerge as highly correlated with early symptoms in BA infants. Furthermore, leveraging these five significant features enhances the interpretability of the machine learning model's performance outcomes for medical professionals, thereby facilitating more effective clinical decision-making.

Proteomics Defines Plasma Biomarkers for the Early Diagnosis of Biliary Atresia.

Early diagnosis of biliary atresia (BA) is crucial for improving the chances of survival and preserving the liver function of pediatric patients with BA. Herein, we performed proteomics analysis using data-independent acquisition (DIA) and parallel reaction monitoring (PRM) to explore potential biomarkers for the early diagnosis of BA compared to other non-BA jaundice cases. Consequently, we detected and validated differential protein expression in the plasma of patients with BA compared to the plasma of patients with intrahepatic cholestasis. Bioinformatics analysis revealed the enriched biological processes characteristic of BA by identifying the differential expression of specific proteins. Signaling pathway analysis revealed changes in the expression levels of proteins associated with an alteration in immunoglobulin levels, which is indicative of immune dysfunction in BA. The combination of polymeric immunoglobulin receptor expression and immunoglobulin lambda variable chain (IGL c2225_light_IGLV1-47_IGLJ2), as revealed via machine learning, provided a useful early diagnostic model for BA, with a sensitivity of 0.8, specificity of 1, accuracy of 0.89, and area under the curve value of 0.944. Thus, our study identified a possible effective plasma biomarker for the early diagnosis of BA and could help elucidate the underlying mechanisms of BA.

Pediatric and adult liver transplantation in Bahrain: The experiences in a country with no available liver transplant facilities.

BACKGROUND: Liver transplantation (LT) is a life-saving procedure for patients with end-stage liver disease and has become the standard and most effective treatment method for these patients. There are many indications for LT that vary between countries and settings. The outcome of LT depends on the available facilities and surgical expertise, as well as the types of liver graft donors available. AIM: To assess the clinical characteristics of patients from Bahrain who underwent LT overseas, and analyze factors affecting their survival. METHODS: In this retrospective cohort study, we reviewed the medical records and overseas committee registry information of all pediatric and adult patients who were sent overseas to undergo LT by the Pediatric and Medical Departments of Salmaniya Medical Complex and Bahrain Defence Force Hospital via the Overseas Treatment Office, Ministry of Health, Kingdom of Bahrain, between 1997 and 2023. Demo graphic data, LT indication, donor-recipient relationship, overseas LT center, graft type, post-LT medications, and LT complications, were collected. Outcomes measured included the overall and 5-year LT survival rate. Fisher's exact, Pearson χ2, and Mann-Whitney U tests were used to compare the pediatric and the adults' group in terms of clinical characteristics, donor-recipient relationship, medication, complications, and outcome. Survival analysis was estimated via the Kaplan-Meier's method. Univariate and multivariate analyses were used to detect predictors of survival. RESULTS: Of the 208 eligible patients, 170 (81.7%) were sent overseas to undergo LT while 38 (18.3%) remained on the waiting list. Of the 170 patients, 167 (80.3%) underwent LT and were included in the study. The majority of the patients were Bahraini (91.0%), and most were males (57.5%). One-hundred-and-twenty (71.8%) were adults and 47 (28.3%) were children. The median age at transplant was 50.0 [interquartile range (IQR): 14.9-58.4] years. The main indication for pediatric LT was biliary atresia (31.9%), while that of adult LT was hepatitis C-related cirrhosis (35.0%). Six (3.6%) patients required re-transplantation. Most patients received a living-related liver graft (82%). Pediatric patients received more living and related grafts than adults (P = 0.038 and P = 0.041, respectively), while adult patients received more cadaveric and unrelated grafts. Most patients required long-term immunosuppressive therapy after LT (94.7%), of which tacrolimus was the most prescribed (84.0%), followed by prednisolone (50.7%), which was prescribed more frequently for pediatric patients (P = 0.001). Most patients developed complications (62.4%) with infectious episodes being the most common (38.9%), followed by biliary stricture (19.5%). Tonsilitis and sepsis (n = 12, 8.1% for each) were the most frequent infections. Pediatric patients experienced higher rates of infection, rejection, and early poor graft function than adult patients (P < 0.001, P = 0.003, and P = 0.025, respectively). The median follow-up time was 6.5 (IQR: 2.6-10.6) years. The overall survival rate was 84.4%, the 5-year survival rate, 86.2%, and the mortality rate, 15.6%. Younger patients had significantly better odds of survival (P = 0.019) and patients who survived had significantly longer follow-up periods (P < 0.001). CONCLUSION: Patients with end-stage liver disease in Bahrain shared characteristics with those from other countries. Since LT facilities are not available, an overseas LT has offered them great hope.

Primary liver transplantation vs transplant after Kasai portoenterostomy in children with biliary atresia: A retrospective Brazilian single-center cohort.

BACKGROUND: Biliary atresia (BA) is the most common indication for pediatric liver trans plantation, although portoenterostomy is usually performed first. However, due to the high failure rate of portoenterostomy, liver transplantation has been advocated as the primary procedure for patients with BA. It is still unclear if a previous portoenterostomy has a negative impact on liver transplantation outcomes. AIM: To investigate the effect of prior portoenterostomy in infants un dergoing liver transplantation for BA. METHODS: This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in Brazil. Patients with BA were divided into two groups: Those under going primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior portoenterostomy. Continuous variables were compared using the Student's t-test or the Kruskal-Wallis test, and categorical variables were compared using the χ2 or Fisher's exact test, as appropriate. Multivariable Cox regression analysis was performed to determine risk factors for portal vein thrombosis. Patient and graft survival analyses were conducted with the Kaplan-Meier product-limit estimator, and patient subgroups were compared using the two-sided log-rank test. RESULTS: Forty-two patients were included in the study (25 [60%] girls), 23 undergoing liver transplantation without prior portoenterostomy, and 19 undergoing liver transplantation with prior portoenterostomy. Patients with prior portoenterostomy were older (12 vs 8 months; P = 0.02) at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores (13.2 vs 21.4; P = 0.01). The majority of the patients (35/42, 83%) underwent living-donor liver transplantation. The group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis (39 vs 11%), but this result did not reach statistical significance. Prior portoenterostomy was not a protective factor against portal vein thrombosis in the multivariable analysis after adjusting for age at liver transplantation, graft-to-recipient weight ratio, and use of vascular grafts. Finally, the groups did not significantly differ in terms of post-transplant survival. CONCLUSION: In our study, prior portoenterostomy did not significantly affect the outcomes of liver transplantation.

Diseases of bile duct in children.

Several diseases originate from bile duct pathology. Despite studies on these diseases, certain etiologies of some of them still cannot be concluded. The most common disease of the bile duct in newborns is biliary atresia, whose prognosis varies according to the age of surgical correction. Other diseases such as Alagille syndrome, inspissated bile duct syndrome, and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction. The majority of these diseases present with cholestatic jaundice in the newborn or infant period, which is quite difficult to differentiate regarding clinical acumen and initial investigations. Intraoperative cholangiography is potentially necessary to make an accurate diagnosis, and further treatment will be performed synchronously or planned as findings suggest. This article provides a concise review of bile duct diseases, with interesting cases.

Analysis of gap length as a predictor of surgical outcomes in esophageal atresia with distal fistula: a single center experience.

PURPOSE: Long-gap esophageal atresia (LGEA) is still a challenge for pediatric surgery. No consensus exists as to what constitutes a long gap, and few studies have investigated the maximum gap length safely repairable by primary anastomosis. Based on surgical outcomes at a single institution, we aimed to determine the gap length in LGEA with a high risk of complications. METHODS: The medical records of 51, consecutive patients with esophageal atresia (EA) with primary repair in the early neonatal period between 2001 and 2021 were retrospectively reviewed. Three, major complications were found in the surgical outcomes: (1) anastomotic leakage, (2) esophageal stricture requiring dilatation, and (3) GERD requiring fundoplication. The predictive power of the postsurgical complications was assessed using receiver operating characteristic analysis, and the area under the curve (AUC) and the cutoff value with a specificity of > 90% were calculated. RESULTS: Sixteen patients (31.4%) experienced a complication. The AUC of gap length was0.90 (p < 0.001), and the gap length cutoff value was ≥ 2.0 cm for predicting any complication (sensitivity: 62.5%, specificity: 91.4%). CONCLUSION: A gap length ≥ 2.0 cm was considered as defining LGEA and was associated with an extremely high complication rate after primary repair.

Glycopyrrolate as an Adjunct in the Management of Anastomotic Leak Following Repair of Esophageal Atresia: A Clinicoradiological Perspective.

Context: Anastomotic leak after primary repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a well-known complication and can represent a challenging clinical scenario. Aims: The present study aimed to evaluate the role of glycopyrrolate as an adjunct in the treatment of anastomotic leak after primary repair of EA Vogt type 3b. Settings and Design: A retrospective study was carried out in our tertiary care teaching institute from January 2015 to December 2022. Materials and Methods: Neonates with EA with distal TEF with primary repair who had developed anastomotic leak, managed by the author(s), were studied. The study included patients with major, minor, and radiological leaks. Glycopyrrolate was administered in the dose of 4 µg/kg 8 hourly. The outcomes of the study were either resolution or progression of the leak. Results: There were 21 patients who were managed with glycopyrrolate in addition to the classical treatment of the anastomotic leak following repair of EA with distal TEF. The male: female ratio was 1:1.1. All the cases had anastomotic leaks with either clinically detectable in the chest tube (15) or radiological leak (6). The leaks were detected early in patients with major leak (mean = 3.2 ± 0.84 days) compared to minor leak (mean =4.9 ± 1.29 days). Radiological leaks were detected in all the neonates on postoperative day 7. In five patients with major leak, there was a negligible reduction in the amount of chest tube output, and were subjected to diversion procedures. There were a total of three deaths out of five in this group. In 10 patients with minor leak, there was complete resolution of anastomotic leak in eight patients (80%); there was one patient each with mortality and diversion procedure. The patients with a radiological leak (6) did not show any deterioration, and they were fed 1-5 days after the esophagogram. Conclusions: Glycopyrrolate may be an advantageous postoperative adjunct in the management of minor and radiological leak after tracheoesophageal repair.

Efficacy and safety of laparoscopic vaginoplasty with peritoneal flaps and cervicoplasty in patients with congenital cervical and complete vaginal atresia: a pilot study.

Background: Hysterectomy places a considerable physical and mental burden on young female patients with congenital cervical and complete vaginal atresia. Thus, it is necessary to develop a method to detach the obstruction and simultaneously preserve the vagina and uterus in these patients. This study sought to evaluate the efficacy and safety of laparoscopic vaginoplasty using peritoneal flaps and cervicoplasty in patients with congenital cervical and complete vaginal atresia. Methods: Between April 2013 and June 2022, nine patients with congenital cervical and complete vaginal atresia at Henan Provincial People's Hospital were enrolled in this prospective study. All patients were treated with laparoscopic vaginoplasty using peritoneal flaps and cervicoplasty. Baseline clinical data (e.g., age and uterus size) were collected. The surgical success rate and adverse events were assessed. Results: The nine enrolled patients had a median age of 15.0 [interquartile range (IQR), 14.0-18.0] years, and five of these patients had pelvic adhesions. The surgeries were successful in all (9/9) patients, with the vagina, uterus, and a normal menstrual cycle being preserved. After a median follow-up duration of 48 months, the neovaginas had a median length of 7.5 cm. Postoperative complications occurred in three of patients and were cured with the appropriate treatment. The five married patients reported being satisfied with their sex life. Conclusions: The study preliminarily demonstrated the efficacy of laparoscopic vaginoplasty using peritoneal flaps and cervicoplasty in patients with congenital cervical and complete vaginal atresia. However, due to the small sample size, lack of a control group, and relatively high incidence of adverse events, further studies are still needed to verify these results. Regardless, our findings establish an approach for preserving both the vagina and uterus for patients with congenital cervical and complete vaginal atresia.

A Female Newborn With Occipital Encephalocele and a Hypoplastic Right Ventricle Secondary to Tricuspid and Pulmonary Atresia: A Case Report.

Encephaloceles and severe cardiac malformations are rare presentations in a newborn. The mechanism of these congenital abnormalities is relatively unknown, but it is hypothesized to be related to genetic, environmental, and maternal risk factors. This case report describes a newborn with an occipital encephalocele associated with severe right ventricular hypoplasia secondary to tricuspid and pulmonary atresia. The patient's maternal risk factors included obesity, type 2 diabetes mellitus, and everyday tobacco use during pregnancy. Education on preconception planning, management, and counseling is essential as a preventative measure in fetal development and is further emphasized in this case.

Successful Stent Implantation Into the Patent Ductus Arteriosus in Complex Cyanotic Congenital Heart Disease.

Birth-associated structural issues with the heart are known as congenital heart disorders or defects. They might alter the heart's regular blood flow. A 10-month-old female child presented to a tertiary care hospital with symptoms of recurrent cyanotic spells with episodes of desaturation a few months after birth. ECG findings depicted a normal sinus rhythm with a right axis deviation along the right ventricular forces. Two-dimensional echocardiography showed a tetralogy of Fallot with pulmonary atresia with a patent ductus arteriosus from the undersurface of the arch with confluent small pulmonary arteries. A coronary wire was passed through the left subclavian artery, and a 4 × 16 mm stent was deployed successfully. After the procedure, the patient's saturation improved, and she was extubated on the table. The patient was on heparin for 24 hours and was started on oral aspirin thereafter. This case was discharged on the third postoperative day and was advised to follow up.

RevCore thrombectomy system for treatment of chronic left external and common iliac vein stent occlusion.

In recent years, deep venous stenting has increasingly become a treatment strategy for post-thrombotic syndrome. Stent thrombosis can occur, resulting in symptom recurrence despite medical therapy, and there are few options available for durable stent patency restoration. We present a case of a 50-year-old male with prior iliocaval reconstruction that experienced recurrent left lower extremity swelling secondary to occlusion of left external iliac and common iliac vein stents during follow-up. Mechanical thrombectomy with the RevCore System and angioplasty was performed. One month later, the patient demonstrated widely patent bilateral iliac vein stents and complete symptomatic resolution. The RevCore System is a feasible alternative for treatment of chronic in-stent thrombosis.

Ductal stenting with bilateral pulmonary artery banding as a life-saving management for hypoplastic left heart syndrome with congenital esophageal atresia: A case series.

We present three cases of hypoplastic left heart syndrome (HLHS) complicated by congenital esophageal atresia and trachea-esophageal fistula (EA/TEF). The standard treatment for HLHS involves a staged surgical approach, eventually reaching Fontan completion. There is no report of patients with both HLHS and EA/TEF, and no established treatment strategy exists for such cases. Given the significant risk of simultaneously operating on HLHS and EA/TEF, we elected to pursue staged repair for each condition separately. Initially, soon after birth, we performed gastrostomy to secure the nutritional pathway for EA/TEF and stabilize breathing. Subsequently, we conducted bilateral pulmonary artery banding (bil-PAB) and ductal stenting for HLHS, as the Norwood operation carried an unacceptably high risk in these patients. Two of these patients were able to transition to home care, while the other patient died during hospitalization due to complications after EA repair. A combination of bil-PAB with ductal stenting for HLHS and staged repair for EA/TEF may provide effective management for patients with both conditions. Learning objective: Hypoplastic left heart syndrome (HLHS) and congenital esophageal atresia (EA) are both life-threatening conditions that require early intervention after birth. There are few reports of patients with both conditions, and no treatment strategy is established. Although the procedure carries a high risk, we successfully performed ductal stenting with bilateral pulmonary artery banding for HLHS, as well as staged repair procedures for EA. Our approach may be a viable strategy for these conditions.