Project description:This case report describes a patient with a 22q11.2 duplication. His features, which include VACTERL association with an esophageal atresia/tracheo-esophageal fistula and a vascular ring, expand the previously described phenotype for this duplication.
Project description:Clinical presentation and management modalities for 585 patients of esophageal atresia and/or tracheo-esophageal fistula reporting to Department of Paediatric Surgery, SMS Medical College, Jaipur over twenty five years (1972-1996) were analyzed in five phases of five years each retrospectively. Over the period of observation, the incidence of new cases as well the number of associated anomalies have shown a steady increase due to availability of modern diagnostic facilities. The operative mortality has shown a progressive decline from 95% early in the series to 40% in last few years. Post-operative anastomotic leaks occurred in 21% of cases, strictures in 8% and recurrent fistula in 1% of cases. The presence of severe associated anomalies, prematurity, pulmonary complications and sepsis still remain the major killers in our setup. Early detection and referral has lead to more infants reaching the specialized centres with less pulmonary complications. In the last decade, the survival rate for infants in Waterston's risk group A has improved to 86% but there is still a substantial difference in the survival rate reported from developed countries. The various factors resulting in poorer results in our set up have been highlighted.
Project description:BackgroundAcute severe mitral regurgitation (MR) associated with cardiogenic shock is a life-threatening emergency. Traditional teaching has focused on the need for emergent coronary angiography and/or intra-aortic balloon counterpulsation in preparation for emergent open-heart surgery for repair/replacement. Unfortunately, emergent open-heart surgery in patients with acute MR complicated by cardiogenic shock is associated with 25-46% perioperative mortality. New devices have provided additional options for stabilization prior to emergent surgery which facilitate improved outcomes.Case summaryWe present two cases of acute severe MR resulting in cardiogenic shock and profound hypoxaemia. TandemHeart® mechanical circulatory support with an oxygenator spliced into the circuit, akin to veno-arterial extracorporeal membrane oxygenation (ECMO), facilitated haemodynamic stabilization and decongestion of the lungs facilitating successful bridge to mitral valve surgery. Successful discharge to home was achieved in both patients with good neurological outcomes and sustained long-term functional recovery at 18 and 14 months, respectively.DiscussionSelective use of the TandemHeart®, with or without ECMO, facilitates management of the critically ill cardiogenic shock patient with acute severe MR.
Project description:BACKGROUND:Tracheo-innominate artery fistula (TIF) is a rare but fatal complication occurring after tracheotomy. Brachiocephalic trunk transection, one of the surgical treatments for TIF, is mostly associated with a full or partial median sternotomy. We describe a case of TIF with continuous bleeding, which was successfully treated with brachiocephalic trunk transection through a collar incision without the need for median sternotomy. CASE PRESENTATION:Case 1. An 18-year-old man was referred to our hospital with bleeding from a tracheal stoma, which had ceased prior to admission. TIF was suspected after examination. Innominate artery transection was performed through a collar incision. TIF was not revealed when we cut the innominate artery anterior wall open; therefore, we opted for preventive surgical intervention. The post-operative course was uneventful, and the patient was asymptomatic at the 3-year follow-up. Case 2. A 14-year-old male patient was admitted to our hospital with bleeding from a tracheal stoma, and TIF was suspected after examination. There was persistent bleeding when the cuff of the tracheotomy tube was deflated. Brachiocephalic trunk transection was performed through a collar incision using balloon occlusion. The post-operative course was uneventful, and rebleeding has not occurred 2?years later. CONCLUSIONS:Brachiocephalic trunk transection without any median sternotomy may offer the benefits of post-operative infection prevention. In patients with suspected continuous bleeding, using a balloon catheter may be a safe and effective method of treatment.
Project description:Atrio-esophageal fistula (AEF) is one of the most devastating complication of radiofrequency catheter ablation (RFCA) of atrial fibrillation (AF) and surgical repair is strongly recommended. However, optimal surgical approach remains to be elucidated. We retrospectively reviewed AEF cases that occurred after RFCA in a single center and evaluated the clinical results of different surgical approach. Surgical or endoscopic repair was attempted in five AF patients who underwent RFCA. Atrio-esophageal fistula and mediastinal infection was not controlled in the patient who underwent endoscopic repair eventually died. Lethal cerebral air embolism occurred two days after surgery in a patient who underwent esophageal repair only. Primary surgical repair of both the left atrium (LA) and esophagus was performed in the remaining three patients. Among these three patients, two underwent external LA repair and the remaining had internal LA repair via open-heart surgery. External repair of the LA was unsuccessful and one patient dies and another had to undergo second operation with internal repair of the LA. The patient who underwent internal LA repair during the first operation survived without additional surgery. Furthermore, we applied veno-arterial extracorporeal membrane oxygenation (VA-ECMO) with artificial induction of ventricular fibrillation in this patient to prevent air and septic embolism and she had no neurologic sequelae. In summary, surgical correction can be considered preferentially to correct AEF. Open-heart surgical repair of LA from the internal side seems to be an acceptable surgical method. Application of VA-ECMO with artificial induction of ventricular fibrillation might be effective to prevent air and septic embolism.
Project description:A newborn presented with a rare combination of esophageal atresia with tracheoesophageal fistula (EA/TEF) and duodenal atresia (DA), which was successfully managed using minimally invasive surgical techniques. The patient was a 1-day-old male for whom passing a feeding tube was infeasible and who had a double bubble sign on radiography. The neonate underwent a thoracoscopic ligation of the tracheoesophageal fistula (TEF) and a laparoscopic duodeno-duodenostomy on the same day, resulting in stabilized vital signs. Ten days after the initial operation, a thoracoscopic esophago-esophagostomy was successfully performed. The patient demonstrated full feeding capability and normal weight gain after the surgeries. The co-occurrence of EA/TEF and DA is a rare and complex anomaly. This case indicates that minimally invasive techniques can effectively manage this condition.
Project description:We report an angiographic image of a 58-year-old woman with profuse bleeding from a tracheo-innominate artery fistula. It may not have been possible to obtain this valuable image if adequate initial resuscitation and an over-inflated tracheostomy tube cuff had not been administered to stop bleeding during an emergency.
Project description:Esophageal atresia with/without tracheo-esophageal fistula is a relatively common malformation, occurring in around 1 in 3500 births. In around half of cases, additional malformations are present, forming either a syndrome of known genetic aetiology, or a recognised association, of which the VACTERL association (Vertebral anomalies, Anal atresia, Cardiac malformations, Tracheo-Esophageal fistula, Renal and Limb malformations) is the most recognised. Recently, microdeletions of the FOX gene cluster at 16q24.1, comprising four genes, FOXF1, MTHFSD, FOXC2 and FOXL1, were reported to cause a phenotype resembling VACTERL association, with vertebral anomalies, gastro-intestinal atresias (esophageal, duodenal and anal), congenital heart malformations, and urinary tract malformations, as well as a rare lethal developmental anomaly of the lung, alveolar capillary dysplasia. This article reviews these new data alongside other genetic causes of syndromic esophageal atresia, and also highlights information from relevant mouse models, particularly those for genes in the Sonic Hedgehog pathway.
Project description:BackgroundAortobronchial fistula (ABF) formation following the rupture of thoracic pseudoaneurysm is a rare clinical entity. Its aetiology includes atherosclerosis, infections, trauma, post-surgery, and post-endovascular aortic repair. The clinical presentation of ABF includes intermittent or massive haemoptysis, acute respiratory distress, hypotension, and even death. These patients require an emergency aortic intervention to stop active haemorrhage. Thoracic endovascular aortic repair (TEVAR) is a less invasive, safe, and effective treatment compared to conventional open surgical repair.Case summaryWe hereby report three cases of ruptured descending thoracic aortic pseudoaneurysms resulting in a fistula formation. The first two cases had tuberculosis as their underlying aetiology, while the third case was the result of previous open post-aortic surgery. All patients presented with massive haemoptysis and were successfully treated by emergency TEVAR and had favourable outcomes.DiscussionThoracic endovascular aortic repair is a rapid, less invasive, and effective treatment for emergency management of ABF. It has more than 85% technical success rates in the reported literature. We had procedural success in all three cases. The short and midterm outcome of ABF following TEVAR is favourable and encouraging.
Project description:IntroductionThe main manifestation of COVID-19 pneumonia is acute respiratory distress syndrome (ARDS), which in some cases can be more severe, requiring Veno-venous extracorporeal membrane oxygenation (VV-ECMO) to ensure hemostasis. Despite support from Veno-venous extracorporeal membrane oxygenation, some patients may remain hypoxemic. One possible therapeutic procedure for these patients is the application of the prone position (PP).ObjectiveThe aim of this study was to investigate the effect of VV-ECMO on arterial oxygenation and compliance of the respiratory system in mechanically ventilated patients with refractory hypoxemia. The secondary objective was to evaluate the safety and feasibility of prone position for ECMO.MethodsWe retrospectively reviewed the electronic records of all 23 COVID-19 patients on ECMO who were placed for the first time in prone position with an average duration of 16 h. Patient characteristics, pre-ECMO characteristics, changes in ventilator/ECMO settings and blood gas analysis before and after PP.ResultsA total of 23 position changes to prone position were performed. Oxygenation and respiratory compliance improved 16 h after adoption of prone position without any accidents during PP.ConclusionsThe use of prone position during Veno-venous extracorporeal membrane oxygenation demonstrated an improvement in oxygenation as well as lung compliance. It is a safe and reliable technique.