Endoscopic management of TEF = tracheo-esophageal fistula.
Published on Feb 25, 2017
Recurrent tracheo-esophageal fistula after surgery for esophageal atresia is a known problem. This video clip demonstrate endoscopic management, i.e. cauterisation of fistula with injection of adhesive glue. Fibrin sealant, or fibrin “glue,” is a unique surgical hemostatic/sealant/adhesive material that is being used with increasing frequency in a variety of surgical situations. In practice, it is a two-component system in which a solution of concentrated fibrinogen and factor XIII are combined with a solution of thrombin and calcium to form a coagulum, simulating the final stage of the clotting cascade. Once the thrombin/calcium is combined with the fibrinogen/factor XIII, a fibrin clot forms in seconds.
Paediatric Surgeon Raisk Shah
Film of: Thoracoscopic Repair of Esophageal atresia
Dr Ravi Kanojia, Published on 23 Feb 2017 – Posted here on 15 march 2017
This is a video showing operative repair of new born esophageal atresia being done thoracoscopically. This is a 2 day old 2.2kg newborn. We call this procedure TREAT
Tof – Tef Repair in Drawings
With Photos, and drawings ect. Learn more
Beautifully illustrated with drawings
1 – Esophageal atresia.
EA is a condition in which the proximal and distal portions of the esophagus do not communicate. The upper segment of the esophagus is a dilated blind-ending pouch with a hypertrophied muscular wall. This pouch typically extends to the level of the second to fourth thoracic vertebra. In contrast, the distal esophageal portion is an atretic pouch with a small diameter and a thin muscular wall; it extends a variable distance above the diaphragm.
TEF is an abnormal communication between the trachea and esophagus. When associated with EA, the fistula most commonly occurs between the distal esophageal segment and the trachea. The distal esophageal segment communicates with the trachea just above the carina. An H-type TEF represents a TEF without EA. It can occur at any level from the cricoid cartilage to the carina, although it usually courses obliquely (with the tracheal end proximal) at or above the level of the second thoracic vertebra.
Background, types of EA and TEF = TOF-OA
Five types of EA and TEF have been described. The most common abnormality is EA with a distal TEF (84%). Isolated atresia with no fistula is the next most common finding (8%), followed by TEF with no atresia (so-called H type) (4%). EA with proximal and distal fistulas (3%) and EA with a proximal fistula (1%) are less common.
Diagram depicting the five variations of oesophageal atresia. (a) Atresia – no fistula (5–10%). (b) Oesophageal atresia with high fistula only (1%). (c) Oesophageal atresia with low fistula only (80–90%).
(d) Oesophageal fistula with low and high fistula (2–3%). (e) H-fistula with no atresia (5–8%). o = oesophagus; s = stomach.
Etiologies and factors.
The etiologies of these anomalies are still largely unknown, but many theories concerning their origins have been proposed. The trachea and esophagus are foregut derivatives. Lateral mesodermal ridges form in the proximal esophagus during the fourth gestational week, and the fusion of these grooves in the midline separates the esophagus from the trachea around the 26th day of gestation.
Notochord abnormalities, desynchronous esophageal mesenchymal and epithelial growth rates, neural crest cell involvement, and incomplete tracheoesophageal separation resulting from a lack of apoptosis are mentioned in some of the theories that have been proposed for EA embryogenesis. Similarly, incomplete tracheoesophageal septation, lateral ridge fusion failure, and tracheal and esophageal proximity have been suggested in explanations of the origin of TEF. In addition, vascular insufficiencies; genetic factors; vitamin deficiencies; drug and alcohol exposures; and viral, chemical, and physical external events may contribute to the development of EA and/or TEF.
Because of the discontinuous esophagus, infants with EA cannot clear their secretions. This defect leads to persistent drooling and aspiration or regurgitation of food after attempted feedings. TEF causes additional complications because of the tracheoesophageal communication. When infants with this anomaly strain, cough, or cry, air enters the stomach through the fistula. As a result, the stomach and small intestine become dilated, elevating the diaphragm and making respiration more difficult. The reflux of food and gastric secretions may also occur up the esophagus and through the fistula into the tracheobronchial tree; this reflux can contribute to pneumonia and atelectasis. Therefore, pneumonia and respiratory distress are common complications.
Abnormal esophageal motility has been observed in children with EA and/or TEF. Controversy often exists as to whether the abnormality was inherently present in the child’s esophagus or if the dysfunction was a result of the surgical treatment. Manometric studies have shown that the motility disorder is present before surgical treatment. Animal studies have also shown that esophageal transection followed by repair does not precipitate disturbances in motility. Discoordinated peristalsis has been reported from the level of the fistula to the stomach in patients with isolated TEF.
Frequency. The international occurrence varies, with estimates ranging from 0.4 to 3.6 cases per 10,000 live births in different regions of the world.
Mortality/Morbidity. Despite an increased number of patients with severe anomalies, survival rates as high as 95% have been reported. In uncomplicated cases, survival rates approach 100%.
The first clinical sign of an infant with EA is maternal polyhydramnios resulting from the infant’s inability to swallow and absorb amniotic fluid through the gut. Polyhydramnios is seen in infants with many diagnoses; only 1 in 12 infants with polyhydramnios have EA. Polyhydramnios is seen in 95% of infants with EA and no fistula and in 35% of patients who have EA with a distal fistula. Increased pressure due to the amniotic fluid accumulation results in a greater number of premature births and neonates with low birth. One third of infants with EA weigh less than 2250 g.
Most infants with EA become symptomatic within the first few hours of life, unlike children with an isolated fistula, who have more subtle symptoms that may not be recognized initially. Excess salivation and fine, frothy bubbles in the mouth and sometimes nose result from an inability to swallow. Any attempts at feeding result in choking, coughing, cyanotic episodes, and food regurgitation. The presence of a fistula increases respiratory complications due to aspiration of food and secretions in the trachea and lungs. Pneumonitis and atelectasis develop quickly in the affected neonate, and rattles heard during respirations are common. Fistulas also allow air to enter the stomach and intestines, leading to abdominal distension. With atresia alone, the abdomen appears scaphoid.
Many anomalies are associated with EA, and 50-70% of children with EA have some other defect.
Once the EA is considered, appropriate diagnostic procedures are necessary. The simplest and quickest diagnostic procedure is the passage of a 10 to 12 French oral tube into the esophagus. If an obstruction encountered (usually in 9 to 13 cm), EA is likely. If the tube passes belong this point, atresia is unlikely. In either case, a radiograph of the chest must be obtained to confirm the position of the tube.
Although ultrasonography has no role in the routine postnatal evaluation of EA and/or TEF, prenatal sonography is a valuable screening tool for EA and/or TEF. The diagnostic accuracy is increased if an anechoic area is present in the middle of the fetal neck; this sign differentiates EA from diseases with possible swallowing impairments.
Provide much information, including findings for EA confirmation and depiction of the side of the aortic arch side, presence of any vertebral or other associated anomalies, and others. Barium studies performed after the surgical placement of a gastrostomy may be used to evaluate the gap length and associated GI abnormalities such as duodenal atresia or malrotation. However, radiographs may not always demonstrate the presence of a fistula.
Findings on posteroanterior and lateral chest images confirm a diagnosis of EA by displaying a coiled nasogastric tube (placed for determination of EA) in the proximal esophageal pouch of a child with EA. Any vertebral anomalies may be visualized, and some cardiac anomalies may be suggested. Aspiration pneumonia, especially in the right upper lobe, and patchy atelectasis are frequently present. Aside from these general findings, the radiographic observations in children with EA and/or TEF vary depending on the type of anomaly present.
The standard approach for repair of an oesophageal atresia
The standard approach for repair of an oesophageal atresia is a right latero-dorsal thoracotomy. If a right aortic arch is diagnosed pre-operatively, a left-sided thoracotomy is recommended.However, if an unsuspected right descending aorta is encountered during surgery, the procedure can be continued in most cases, establishing the anastomosis on the right of the aortic arch.
The baby is positioned on the left side, stabilized with sandbags and fixed to the table with adhesive bands.The right arm is abducted without undue tension. Mild anteversion helps to reduce the risk of traction injury to the brachial plexus. The elbow is flexed to 90°, and the forearm is best tied to a transverse bar mounted over the head of the child with soft slings. Care must be taken that no part of the body is submitted to pressure during the procedure. Exposed sites must be well padded. Soft pillars may be placed between the knees and underneath the feet, or the limbs wrapped with cotton wool, which protects against heat loss at the same time. A folded towel under the left side of the chest improves exposure and facilitates access in particular to the deeper structures.
A slightly curved skin incision is placed 1 cm below the tip of the scapula from the midaxillary line to the angle of the scapula. Some surgeons prefer a vertical skin incision in the midaxillary line for cosmetic reasons.A major advantage in neonates is the possibility of employing a muscle sparing technique – due to their soft and mobile tissue layers. Only small flaps of skin and subcutaneous tissue are raised around the incision. The latissimus dorsi muscle is mobilized by cutting through the anterior fascial attachment. It is then lifted off the thoracic wall and retracted posteriorly together with the thoracodorsal nerve, which runs on its deep surface following the posterior axillary line.When the latissimus muscle is rectracted, the border of the serratus anterior muscle is mobilized along its origin from the tip of the scapula to the sixth rib and retracted up and forwards simultaneously with the scapula.
The intercostals muscles are divided along the upper border of the fifth rib.When the parietal pleura is exposed in one spot, a tiny moist cotton swab mounted on an artery forceps is used to sweep it off the thoracic wall for an extrapleural approach.As soon as possible, a rib spreader is inserted and opened stepwise with care. For continuation of the pleural stripping towards the dorsal mediastinum, the use of two soft pledgets is recommended, one to hold the already reflected pleura under mild tension by pressing it towards the dorsal mediastinum, the other to proceed with the dissection. An inadvertent tear in the pleura can be closed with a fine (6/0) monofilament absorbable suture.
The azygos vein is mobilized with right-angled forceps and divided in between two ligatures (4/0 Vicryl). The right vagus nerve is identified, which runs along the lateral border to the upper pouch and accompanies the tracheo-oesophageal fistula towards the lower oesophagus. The lower oesophagus is usually rather thin and hypoplastic. Extreme care must be taken to avoid any trauma to the delicate tissue. Handling and squeezing the oesophageal wall with forceps should be restricted to an absolute minimum. Preservation of all vagal fibres supplying the lower oesophagus is also aimed for. Denudation invariably entails a significant motility disorder and may cause severe gastro-oesophageal reflux.
Right-angled forceps are passed behind the distal oesophagus and a vascular sling is placed around it in order to pull it away from the trachea. This facilitates identification of tracheo-esophageal fistula, which is now freed from surrounding tissue. Traction sutures are then placed at the tracheal and oesophageal ends of the fistula, and one additional stay suture nearby holds the lower oesophagus.
At this stage, the fistula is divided and closed with a continuous absorbable monofilament 6/0 suture. Some authors prefer interrupted stitches, others apply transfixation stitches. The level of division must be as close to the trachea as possible without risking a narrowing of the airway. Since most fistulas run obliquely upwards, a small residual pouch frequently remains in the trachea. The fistula closure is tested for an air leak by watching out for air bubbles during forceful ventilation after filling warm saline solution into the chest. At this stage it is advisable to temporarily relieve the lung from the continuous retraction and achieve through careful ventilation cycles a full expansion of all collapsed areas.
The upper pouch is often retracted into the neck. Asking the anaesthetist to push on the Replogle tube serves to advance the upper pouch into the operative field.Traction sutures are placed on either side of the pouch to assist mobilization. Dissection of the oesophagus from the trachea is most challenging because they are adherent to each other by an intervening firm connective tissue layer. Sharp scissor dissection is required taking extreme care to avoid any accidental penetration into either organ. Anterior and lateral aspects of the upper pouch are easily freed using pledgets. If an upper fistula is encountered, it is transected close to the oesophagus and closed on both sides with interrupted monofilament absorbable 6/0 sutures. Contrary to the lower oesophagus, the upper pouch has an excellent blood supply and can be dissected up to the thoracic inlet if necessary. Thus, if a large gap exists, further dissection of the upper oesophagus is preferable to extensive mobilization of the lower segment which involves the risks of ischaemia and subsequent dysmotility.
After the upper oesophageal pouch is mobilized, both segments are approximated to see whether an end-to-end anastomosis is possible.
Opening of the upper pouch for the anastomosis should be well centred at its lowermost point. This is best achieved by incising the pouch exactly over the tip of the fully advanced Replogle tube.An asymmetric opening results in an uncentred anastomosis, potentially leading to lateral pre-anastomotic outpouching. The upper pouch is opened by a horizontal incision, which results in a fish-mouth-shaped aperture, adapted to the diameter of the lower oesophagus.
The end-to-end anastomosis is fashioned with interrupted absorbable 6/0 sutures. The first two stitches are placed on either side. The posterior wall needs two or three additional sutures.Meticulous care must be given to take sufficiently large “bites” of muscular tissue together with the mucosal layer. The latter tends to retract upwards in the upper pouch as soon as it is opened. Once all posterior wall sutures are placed, the oesophageal segments are gently pulled together, and the sutures are tied on the mucosal surface.
Thereafter, a 5F silastic feeding tube – the connection hub of which has been cut off – is sutured with the cut end to the tip of the Replogle tube,which is then withdrawn by the anaesthetist until the feeding tube appears outside the mouth. The distal end of the feeding tube is passed into the stomach. The tubeserves for postoperative gastrointestinal decompression and early feeding, and also functions as transanastomotic splint for drainage of saliva.
The anterior aspect of the anastomosis is completed in a similar way as described above with three or four stitches, this time tying the knots on the outside of the oesophageal wall.
The goal of a tension-free end-to-end anastomosis can be achieved with this technique in most cases of oesophageal atresia with a distal fistula. If the tension appears to be too much despite mobilization of the upper pouch up to the thoracic inlet, further length may be gained with a circular myotomy in the upper pouch according to Livaditis. This is achieved by introduction of a 8F balloon catheter into the upper pouch transorally, which is transfixed at the lower end of the pouch with a 4/0 monofilament traction suture and the balloon is blown up until it fills the pouch. The muscle layer is then divided above the balloon approximately 1 cm cranial to the future anastomotic line, either in a circular or in a spiral fashion. The mucosal layer of the upper pouch is rather thick so that mucosal tears can usually be avoided with careful dissection.The upper pouch can be lengthened by 5–10 mm by this method, which may suffice to create an anastomosis without undue tension. Development of a pseudodiverticulum (outpouching of the mucosa through the established gap in the muscle layer) after circular myotomy has been described.
Another way to reduce inappropriate tension on the anastomosis is to fashion a mucosal-muscular flap from a larger upper oesophagus. A right-angled incision is made in one half of the upper pouch. The flap thus created is turned by 90° so that the vertical cut surface faces downwards. It is then rolled into a tube.However, the gain in length results in a reduction in diameter.
If a satisfactory dorsal wall anastomosis can be established, but undue tension arises in the anterior half, a right-angled flap in the corresponding part of the upper pouch without tubularization may bridge the gap and result in a safe anastomosis. The thoracic cavity is irrigated with normal saline. A soft drain is introduced via a separate intercostals stab incision and the tip placed near the anastomosis. Before closure, the lungs are fully expanded by forced ventilation until all collapsed regions are well aerated again.
The ribs are approximated with two or three pericostal sutures. Latissimus dorsi and serratus anterior muscles are allowed to fall back into their original positions and are sutured to their fascial insertion sites with one or two 3/0 absorbable sutures each. The subcutaneous fat is readapted with 5/0 absorbable sutures including the corium.This technique approximates the skin perfectly in most cases so that separate skin sutures are not necessary. The incision is simply approximated with adhesive strips. In those cases in whom wound margin adaptation remains unsatisfactory, a continuous subcuticular monofilament 5/0 suture is applied,which is pulled after a few days.
The patient is managed by a team approach that includes surgeon, neonatologist, nurses, and respiratory therapists. In a near-term baby with an uncomplicated anastomosis, the anesthetic effects are allowed to wear off and extubation is achieved within 24 h. Fentanyl is used for postoperative pain, either by bolus or continuous drip. Some prefer to keep the child heavily sedated or even paralyzed for a few days, especially when the anastomosis has been done under a lot of tension, in which case extubation may be delayed for up to 6 or 7 days. Neck flexion has also been advocated to decrease tension on the anastomosis. We keep the tip of the Replogle tube above the anastomosis as marked intraoperatively and under continuous suction. Some remove the Replogle after a few days if there is minimal drainage, indicating passage of saliva through the anastomosis. Parenteral nutrition is started as soon as possible and enteral feedings are initiated through the silastic feeding tube.
The extrapleural chest tube is kept on underwater seal drainage and gentle (10 cm H2O) suction added for the first 24 h. Usually only a minimal amount of serous drainage is noted. In a stable extubated baby, we obtain a contrast esophagram and UGI series under fluoroscopy 5–7 days postoperatively. After removing the Replogle, we usually start this study with a non-ionic isoosmolar water-soluble agent, then switch to dilute barium if there is no aspiration or anastomotic leak. We observe the swallowing reflex, esophageal motility, anastomotic site and distal esophagus. Then, if the baby is tolerating the procedure well, we fill the stomach enough to look for GER and assess gastric emptying and the position of the ligament of Treitz. It is normal for the esophageal anastomosis to appear narrowed and for the upper esophagus to appear dilated, but there should be no stasis.
As long as there is no leak and good swallowing, feedings are initiated after this study and will help to gradually dilate the anastomosis and lower esophagus. The chest tube is removed the next day and antibiotics are stopped if this is not already done. Oral feedings often progress slowly because of poor sucking or swallowing reflexes. The silastic nasogastric tube is used to complete each feeding, after the child has been offered the bottle.
The infant may be discharged when feeding well and gaining weight, occasionally with the feeding tube still in place to ensure adequate intake. Parents are warned about the signs of complications such as reflux, tracheomalacia, anastomoticstricture, and recurrent fistula. We discharge all babies on H2-blockers until a pH probe is done at 6 months of age. Patients are followed up frequently in the first year of life, and then once or twice a year at least until school age and preferably until adulthood. It is very important to explain to the parents that there will be some permanent scarring at the anastomosis, which prevents normal distensibility of the esophagus at this site. We therefore recommend pureed food up to 12–18 months, and then only minced food until 5 years of age when the child has learned to chew well before swallowing, and has adequate teeth to do so. All those caring for such children have seen patients admitted with an impacted foreign body at the anastomotic site despite a normal appearance of the anastomosis on contrast studies. This might be food, often a piece of meat or popcorn, or a foreign body.
Although most term babies do very well after esophageal atresia repair, some even going home by 1 week of age, the potential complications are numerous. These can be related to associated problems such as prematurity and cardiac defects, to the malformation itself, or to its treatment. Our discussion will focus on the latter. These may be divided roughly into early (<30 days), intermediate (1–3 months) and late complications.
Anastomotic leaks occur in 5–10% and can be suspected by the presence of frothy saliva in the chest tube drainage. Small extrapleural leaks that are well drained by the tube are treated with continued upper pouch suctioning and antibiotics and usually seal spontaneously. Some surgeons even start feedings despite a leak, but we prefer to wait until radiological resolution. Beware of anastomotic stenoses, which often develop within a few weeks after a leak. A complete hemithorax “white-out” or a massive pneumothorax is usually caused by a major leak or a total anastomotic disruption. Breakdown of the tracheal suture line should also be considered. The child can rapidly deteriorate and necessitate more chest tubes and emergency thoracotomy.
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From this above website link, learn more about the 5 subjects found below.
Find more Beautiful illustrations on the subjects found under here, This was a truly fantastic find, and one you can see, read and understand a lot from.
Congenital Lobar Emphysema.
Congenital Cystic Adenomatoid Malformation.
Cont, click on link below to see way more (same website as above)
EUROPEAN REFERENCE NETWORK WHICH FOCUSES MAINLY ON EA
ERN on inherited and congenital abnormalities (ERNICA)
ERNICA addresses congenital malformations and diseases that appear early in life and require multidisciplinary care and long-term follow-up and examines the transition to adulthood. The network is organized around two main work streams in line with ORPHANET classifications and ICD10. One work stream deals with malformations of the digestive system and the other deals with malformations of the diaphragm and abdominal wall. In the latter work stream, there are working groups covering malformations of the esophagus and a group working on gastroenterological and intestinal diseases. This group also includes a sub-group specializing in intestinal failure. Each working group has its own disease-specific task forces. For some of these rare diseases, mortality rates can be as high as 50%. ERNICA aims to improve the quality of care that patients receive and to reduce the long-term impact of these rare diseases in infants. The network will facilitate research collaborations with the power to develop evidence-based clinical guidelines. Access to new surgical techniques and treatments will also be improved. ERNICA is a meeting place for national patients’ associations and caregivers, including nurses and other professions committed to improving patient outcomes, For some of these rare diseases, mortality rates can be as high as 50%.
NETWORK COORDINATOR Professor Rene Wijnen Erasmus Medical Center Rotterdam, The Netherlands
Gut is a leading international journal in gastroenterology and hepatology and has an established reputation for publishing first class clinical research of the alimentary tract, the liver, biliary tree and pancreas. Gut delivers up-to-date, authoritative, clinically oriented coverage in all areas including articles by leading authorities, reports on the latest treatments for diseases, reviews and commentaries.
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Gut is an official journal of the
Guidelines on the use of oesophageal dilatation in clinical practice
Oesophageal dilators or bougies have been used since the Middle Ages. Early bougies were made of natural materials and were used to disimpact food boluses by pushing them “blindly” into the stomach. The technique of oesophageal dilatation has evolved considerably in recent years. A range of purpose built dilators is now available, and with present day diagnostic techniques it is possible to select a dilator and dilatation technique appropriate to the clinical setting.
The relatively low morbidity and mortality of oesophageal dilatation has encouraged its widespread use. Despite this wealth of clinical experience however, the practice of oesophageal dilatation has been subject to surprisingly few controlled studies. The purpose of these guidelines is to highlight areas of good practice and promote the use of standardised protocols within and between centres.
Esophageal lung – A rare bronchopulmonary foregut malformation
Esophageal lung is a rare variety of communicating bronchopulmonary foregut malformation characterized by a fistula between an isolated portion of respiratory tissue and esophagus or stomach. It may involve the entire lung or one of the pulmonary lobes. Only 20 cases have been reviewed in 2011. Fifty percent of cases are associated with a tracheoesophageal fistula.
A six month old girl, who was operated for an extrapleural repair of TEF type C on day 2 of life, came with complaints of persistent cough and lower respiratory tract infections since past 2 months. 2D echocardiography showed a minor cardiac anomaly (tiny patent foramen ovale), which did not require any cardiological intervention. She underwent an upper GI contrast study that showed an outpouching from the lower third of esophagus. However, the outpouching seemed to be much lower down than the expected site of esophageal anastomosis. Hence, a diagnosis of recurrent TEF seemed less likely. Another possibility of a second lower pouch fistula, which could have been missed at the time of first surgery, was also thought of. She underwent upper GI scopy where the suspected fistula was visualized. An attempt was made to tackle the fistula endoscopically and ligate it with vascular clips. However, the symptoms persisted and a repeat dye study showed persistent out pouching.
A contrast enhanced computed tomogram (CT scan) showed a collapsed lower lobe communicating with the esophagus. It had no communication with the trachea-bronchial tree and hence a diagnosis of esophageal lobe was made. A right posterolateral thoracotomy with lower lobectomy (excision of esophageal lobe) was planned. The lower lobe bronchus was found to communicate with the esophagus. There was no communication with the parent tracheobronchial tree. The lobe received its blood supply from the pulmonary vessels. The lobe was excised and the esophagus was closed. The histopathology report showed lung tissue with lymphocytic infiltrate and the communicating bronchus showed presence of cartilage Postoperative dye study was normal. Patient is asymptomatic at follow up of 2 months with weight gain of 800 g.
A – Dye study showing lower esophageal-bronchial communication.
B – Repeat dye study showing persistent communication.
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A rare case of esophageal lung in a neonate
We report a rare case of esophageal lung in a neonate who presented with repeated chest infections and respiratory distress. Chest radiograph revealed increased opacification of the right lung with reduced lung volume and air bronchograms. Further evaluation with computed tomography (CT) showed the presence of only left mainstem bronchus at the tracheal bifurcation. Right mainstem bronchus originated from distal esophagus and aerated the right lung. Nasogastric tube was inserted into the stomach with injection of small amount of dilute barium through it, which established the communication of right mainstem bronchus with esophagus. Advanced CT scan imaging by virtual bronchoscopy and volume rendering further delineated the anatomical abnormality precisely prior to surgery. Surgical findings confirmed the diagnosis.
Esophageal lung is an extremely rare type of bronchopulmonary foregut malformation where a main stem bronchus anomalously arises from the esophagus instead of the trachea. Less than 20 cases have been reported, and most of these are in association with esophageal atresia. Most cases are treated with detachment of the esophageal bronchus with repair of the esophagus and resection of the hypoplastic lung. Described herein are the presentation, diagnostic workup, and management of 2 such cases of esophageal lung.
Type II congenital pulmonary airway malformation in an esophageal lung
A seven-month-old girl, born prematurely (birth weight 1000 g) from a twin pregnancy, was admitted to hospital due to recurrent pneumonia and atelectasis. She experienced cough and respiratory distress during feeding. The right hemithorax was smaller than the left, with diminished breath sounds and dullness. Chest x-rays revealed decreased lung volume and multiple radiolucent images in the right lung, as well as overdistention of the left lung. An esophagogram revealed three bronchial branches arising from the lower one-third of the esophagus, corresponding to the right lung and ending in a cul-de-sac. A diagnosis of esophageal lung was established. On bronchography, the right lung was absent and the trachea only continued into the left main bronchus. Echocardiography and angiotomography revealed agenesis of the pulmonary artery right branch. The surgical finding was an esophageal right lung, which was removed; the histopathological diagnosis was type II congenital pulmonary airway malformation in an esophageal lung.
The esophageal lung or esophageal bronchus is characterized by an aberrant bronchus or lung that arises from the esophagus, with total absence of ventilatory function in the involved pulmonary tissue (1–5). The present report describes an unusual case of a type II congenital pulmonary airway malformation (CPAM) that occurred in an esophageal lung
The patient was a seven-month-old girl born after 34 weeks’ gestation in a twin pregnancy, with a birth weight of 1000 g. In the neonatal period, she developed pneumonia and required mechanical ventilation for 40 days, and was discharged eight days later. She was hospitalized on three more occasions due to pneumonias and was sent to the Hospital de Pediatría, Centro Médico Nacional Siglo XXI (Mexico DF) for evaluation and treatment.
On admittance, her weight was 2900 g, oxygen saturation was 92% on room air, with respiratory distress, asymmetrical thorax due to a smaller size of the right hemithorax. She arrived without an orogastric tube and, thus, previous oral feeding was assumed, but she experienced an episode of choking after trying to initiate by oral route.
A chest radiograph revealed a pencil-like tapering of her tracheal air column, and the right lung was smaller and with multiple radiolucent images compared with the left lung, which was overdistended. Bronchoscopy revealed 20% subglottic stenosis and progressive diminution of tracheal lumen and, thus, the carina could not be visualized. Endoscopy showed incompetent hiatus. In contrasted images, the barium swallow and a Valsalva manoeuvre revealed a three-branch ramification emerging from the lower one-third of the esophagus, which ended in a cul-de-sac, establishing the diagnosis of esophageal lung.
National Esophageal Atresia Register
National Esophageal Atresia was created in 2008 by the National Reference Center for Esophageal Congenital Abnormalities created in 2006. Primary goal was estimation of live birth prevalence in France. A national network of surgeons and pediatricians was initiated and entire teams dealing with esophageal atresia accepted to participate in an exhaustive national register. A questionnaire was validated by a national committee and data were centralized in our center. Scientific exploitation showed that such database is useful for health authorities as for medical professionals. Live birth prevalence in France is at 1.9/10,000 births. Prenatal diagnosis is more common but its effect on prevalence is not yet fully understood. Associated congenital abnormalities are frequent and major malformations with termination of pregnancy can influence prevalence.
Congenital Anomalies of Esophagus Treatment & Management
Dec 07, 2015
All children with esophageal atresia (EA) and many with congenital stenosis require surgical intervention. The diagnosis of EA or tracheoesophageal fistula (TEF) can be made prenatally and after birth by clinical signs and supportive findings on imaging studies. Prenatally, an ultrasonographic finding of a small or absent stomach bubble suggests EA with 42% sensitivity. Prenatal magnetic resonance imaging (MRI) was used to evaluate the esophagus of fetuses with a small or absent stomach bubble on ultrasonographic evaluation. Positive findings on prenatal MRI had a sensitivity of 100% and specificity of 80%. After birth, failure of passage of a rigid radiopaque 10F catheter from the mouth to the stomach suggests EA. The diagnosis is typically confirmed with plain radiography.
The presence of air in the stomach and intestines indicates EA with a distal fistula; the absence of abdominal gas suggests pure atresia, EA with a proximal fistula, or on rare occasions, EA with an occluded distal fistula. A small upper esophageal pouch is suggestive of a proximal fistula, and the presence of a proximal TEF can be confirmed with fluorography, endoscopy, bronchoscopy, or upper esophageal contrast studies. An isolated TEF may be detected by barium esophagography, cinefluoroscopy, bronchoscopy, or esophagoscopy. Because of the risk of aspiration, the use of contrast for visualization of congenital esophageal anomalies must be approached with extreme care and performed only by an experienced radiologist.
Although a diagnosis of EA or TEF is no longer considered a surgical emergency, because of improvements in neonatal intensive care, respiratory problems may still develop and rapidly progress. A period of 24-48 hours between diagnosis and surgical repair allows for a thorough assessment of the neonate and treatment of any pulmonary complications. In general, vigorous infants weighing more than 1300 g and without pulmonary insufficiency or major associated anomalies should be considered for repair.
Historically, prognostic risk classification was based on birth weight, the presence and severity of pneumonia, and congenital anomalies for infants with EA. Subsequently, ventilator dependence and severe anomalies, not birth weight, have been linked to mortality. Regardless of the classification used, stronger infants with fewer concomitant disorders have lower risks associated with the surgical repair.
Intervention for esophageal stenosis, webs, and tracheobronchial remnants is indicated according to the diagnosis and the presence of symptoms. The selection and timing of surgical treatment for congenital esophageal anomalies depend on the type of lesion, the presence and severity of associated anomalies……..
A method to detect ……
A method to detect different disease-causing viruses on surfaces has now been developed
Viruses are the cause of many of the gastrointestinal and respiratory infections.
Viruses cause a major proportion of human infections, especially gastroenteritis and respiratory infections in children and adults. Indirect transmission between humans via environmental surfaces may play a role in infections, but methods to investigate this have been sparse.
July 12th 2016
New method reveals where viruses hide
A method that can detect different disease-causing viruses on surfaces like countertops and fixtures, has been developed in a project at the National Food Institute, Technical University of Denmark. The method can be used to test for the presence of viruses in the environment in places like childcare centres, nursing homes, hospitals and food production premises. Test results make it possible to focus cleaning on the places that are the biggest sources of infection. Viruses are the cause of many of the gastrointestinal and respiratory infections, which cause thousands of Danes to get sick each year. In addition to direct transfer of infection between people many viruses are probably transferred via infected surfaces such as hands, sinks and tables. However, effective methods to test this presumption have been limited.
A method to detect different disease-causing viruses on surfaces has now been developed in a project at the National Food Institute.
Method makes targeted cleaning possible
The method makes it possible for e.g. childcare centres, nursing homes, hospitals and food production premises to test for the presence of viruses. The test results make it possible to focus cleaning on the surfaces that are at greatest risk of transmitting infection.
Among the food sources that most often cause norovirus in people is food prepared in institutions, restaurants or catering companies. This is partly because food handlers themselves are infected or via their relatives carry the virus while they are working, and partly because they return to work after having recovered from symptoms, although they still in fact are carriers of the infection by continuous viral shedding.
The test method from the National Food Institute can be used e.g. in the regular cleaning control system of the institution or food establishment, or after an employee or a guest has vomited to check whether the subsequent cleaning has been sufficient to stop the virus from spreading.
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What is SIBO?
Click on the RED underlined Words to look at that link
Small intestinal bacterial overgrowth occurs when the bacteria in our gut get out of balance and overgrow. I often say, “Too much of a good thing can be a bad thing.” How do we get too much of some bacteria over others? This can manifest in several different ways, and often occurs in those eating a diet high in sugar, alcohol and refined carbohydrates. Certain strains of bacteria feed off of refined carbohydrates and break them down into short-chain fatty acids, creating gas and causing bloating.
Another strain of bacteria can break down bile salts before your body has a chance to use them. Bile salts are crucial for the breakdown of fats; without them, the end result is fat malabsorption or diarrhea.
Finally, a third type of bacteria can produce toxins that damage the lining of the small intestine. This prevents your body from absorbing the nutrients you need, much like what we see with a leaky gut.
April 13, 2015
I will say this from the start, if there is a medication out there that will help a patient fix SIBO and the daily, often debilitating symptoms that they are experiencing, I am first in line. I also want people to recover – long term. Though I have seen xifaxan and the combination of xifaxan and neomycin work for some people, it seems like these are successful at knocking down SIBO long term for only a very small percentage of people.
Prior to meeting with a new SIBO patient I spend around 45 minutes combing through all the labs, intake paperwork and health history timeline in preparation for our intake session. It is extremely common for me to see a new patient who has undergone four and five, or more rounds of antibiotics, trying to knock out SIBO.
Interesting website, with an interesting video