Primary Esophageal Repair Research Articles

P-07: Vascular Anomalies Associated With Esophageal Atresia and Tracheoesophageal Fistula: Incidence, Clinical Presentation, Diagnosis and Consequences

Vascular anomalies may be associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF). Our main objective is to report their incidence in a cohort of EA/TEF patients while describing clinical presentation, diagnosis and consequences. The secondary objective is to determine the diagnostic value of esophagram in the diagnosis of aberrant right subclavian artery (ARSA). All patients born with EA/TEF from 2005 to 2013 were studied. Preoperative echocardiography reports, surgical description of primary esophageal repair and esophagram were retrospectively reviewed. Age at diagnosis, discovery mode, clinical presentation and need for surgical correction of the vascular malformation were noted. 76 of 86 children with EA/TEF were included. Fourteen children (18%) had a vascular malformation. The incidence of right aortic arch (RAA) and ARSA was 6% (5/76) and 12% (9/76) respectively. Respiratory and/or digestive symptoms occurred in 9 of them. Long gap EA and severe cardiac malformations requiring surgery were both significantly associated with vascular anomalies (p < 0.05). We reviewed 254 esophagrams; 40% were inconclusive for the detection of vascular anomalies. The diagnosis of vascular malformation was missed in four patients with a long gap EA. The sensitivity of esophagram for the diagnosis of ARSA was 66%, the specificity was 98%, the negative predictive value 95%, and the positive predictive value 85%. ARSA and RAA have an incidence respectively of 12% and 6% in EA/TEF patients. Echocardiography and esophagram are effective but their sensitivity is not optimal for the diagnosis of ARSA. A CT-angioscan is recommended when esophageal stenting is indicated.

Vascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula

To report the incidence of congenital vascular anomalies in a cohort of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) while describing the clinical presentation, diagnosis, and consequences, and to evaluate the diagnostic value of esophagram in diagnosing an aberrant right subclavian artery (ARSA). All patients born with EA/TEF between 2005 and 2013 were studied. Preoperative echocardiography reports, surgical descriptions of primary esophageal repair, and esophagrams were reviewed retrospectively. Of the 76 children born with EA/TEF included in this study, 14 (18%) had a vascular malformation. The incidence of a right aortic arch (RAA) was 6% (5 of 76), and that of an aberrant right subclavian artery (ARSA) was 12% (9 of 76). RAA was diagnosed in the neonatal period by echocardiography (4 of 5) or surgery (1 of 5), and ARSA was diagnosed by echocardiography (7 of 9) or later on the esophagram (2 of 9). Respiratory and/or digestive symptoms occurred in 9 of the 14 patients with vascular malformation. Both long-gap EA and severe cardiac malformations necessitating surgery were significantly associated with vascular anomalies (P<.05). The sensitivity of the esophagram for diagnosing ARSA was 66%, the specificity was 98%, the negative predictive value was 95%, and the positive predictive value was 85%. ARSA and RAA have an incidence of 12% and 6% respectively, in patients with EA/TEF. A computed tomography angioscan is recommended to rule out such malformations when stenting of the esophagus is indicated, before esophageal replacement surgery, and when prolonged (>2 weeks) use of a nasogastric tube is considered.

Case of von Recklinghausen′s neurofibromatosis with esophageal neurofibroma

Neurofibromatosis (NF) is a term that has been applied to a variety of related syndromes, characterized by neuroectodermal tumors arising within multiple organs and autosomal-dominant inheritance. NF-1, known as well as Recklinghausen's disease, is the most common type of the disease accounting 90% of the cases. A case of 50-year-old patient with NF is presented with a mass in the left side of the neck since 1-year and progressive dysphagia since 6 months worse for solids than liquids. Intraoperatively a large solid mass was found occupying the left side of the neck pushing the trachea and esophagus to the right side, infiltrating a portion of the wall of esophagus. The mass was resected en bloc along with a portion of the wall of esophagus, and primary esophageal repair was done. Histology revealed plexiform neurofibroma.

Transthoracic extraction of impacted denture at mid esophagus in a 75-year-old patient

A 75-year-old male patient presented with history of dysphagia following inadvertent passage of loose dentures into the esophagus. Patient could only swallow liquids thereafter and had persistent pain in the throat. Upper gastrointestinal endoscopy confirmed the presence of denture in mid esophagus at approximately 22-25 cm from incisors but the denture could not be removed as it had got impacted in the esophagus. Thoracotomy and esophagotomy was planned because repeated endoscopic removal was unsuccessful. Right posterolateral thoracotomy was performed through the fifth intercostal space and longitudinal esophagotomy in the mid esophagus was done to extract the impacted dentures followed by primary repair of the esophagus. The patient recovered well and he was discharged on the eighth postoperative day.

Clinical outcomes after repair of left atrial esophageal fistulas occurring after atrial fibrillation ablation procedures

The initial experience with left atrial esophageal fistula (LAEF) secondary to atrial fibrillation (AF) ablation procedures revealed a near-universal mortality. A comprehensive description of the principles of LAEF repair in the modern era and its resulting impact on morbidity and mortality are lacking in the literature. To describe the presentation, surgical management, and outcomes of patients with LAEF. A retrospective cohort analysis of 29 patients was performed, including previously unpublished cases of surgically repaired LAEF from 4 institutions (n = 6), and all published cases of surgically repaired (n = 16) or stented (n = 7) LAEF. The mean age was 55 ± 13 years, and 75% were men who were undergoing radiofrequency energy catheter ablation (n = 26), cryoablation (n = 1), high-intensity focused ultrasound ablation (n = 1), and surgical mini-MAZE procedure (n = 1) and presented 30 ± 12 days postablation procedure. Overall, 55% of the patients receiving an intervention for LAEF died (41% surgical repair; 100% stent). Patients who did not receive primary esophageal repair were more likely to experience postoperative complications, including mediastinitis, need for percutaneous endoscopic gastrostomy (PEG) feeds, esophageal stent, or death (P = .05). In addition, interposing tissue between the repaired esophagus and the left atrium resulted in fewer postoperative complications (P = .02). While improved relative to initial reports, mortality associated with LAEF remains high after corrective intervention. Primary esophageal repair with the placement of tissue between the repaired esophagus and the left atrium may result in lower morbidity and mortality.

Agenesis of azygos vein in a case of esophageal atresia and tracheoesophageal fistula.

A 2-day-old female neonate, weighing 2.7kg, presented with salivation, frothing, and choking at feeding attempts. There was a history of polyhydramnios in mother. The baby was a product of non-consanguineous marriage and delivered at term by normal vaginal delivery in a private hospital. General physical examination was unremarkable. There were no visible associated anomalies in the baby. Chest and upper abdominal radiograph showed feeding tube curled just beyond thoracic inlet and a big gastric shadow. Laboratory investigations were within normal limits. Patient underwent right thoracotomy which revealed agenesis of azygos vein (Fig. 1). Primary esophageal repair was done with mild anastomotic tension. Postoperative recovery was uneventful. Echocardiogram done postoperatively showed structurally and functionally normal heart. Patient was discharged in good general condition.

Boerhaave’s syndrome or spontaneous perforation of the oesophagus

Spontaneous perforation of the oesophagus, also called Boerhaave’s syndrome, is a barogenic rupture caused by a sudden rise in intraluminal pressure, usually in the distal oesophagus. Early diagnosis of spontaneous oesophageal perforation is critical to the survival of the patient. Sudden and very intense chest or epigastric pain after an abrupt increase of oesophageal intraluminal pressure is the most characteristic clinical symptom and should raise suspicion for Boerhaave’s syndrome. Plain radiographs demonstrate the presence of free air in the mediastinum, subcutaneous emphysema or air subdiaphragmatically, as well as pleural effusions. Upper gastrointestinal series reveal leakage of the contrast and confirms the location of the perforation. Similarly, chest computed tomography demonstrates the presence of an air-fluid level, pneumomediastinum and pleural effusions and, more importantly, the complications related to oesophageal perforation. Oesophageal perforation is associated with a high mortality rate, suggested to be approximately 2% for every hour after initial presentation. More specifically, diagnosis and intervention within the first 12 hours results in 80-90% survival, which falls to 70-75% between 12-24 hours; after 24 hours, survival decreases to less than 50%, and after 48 hours to less than 10%. Operative treatment of Boerhaave’s syndrome mainly includes a complete debridement and lavage of infected and necrotic tissues with wide drainage of the mediastinum and pleural cavities and either primary oesophageal repair and reinforcement (if diagnosed early) or T-tube diversion or resection (if diagnosis is delayed). Early confirmation of the diagnosis is very important because it allows earlier support of the critically ill patient and prompt operative intervention when necessary. Eventful outcome of patients is related to these

A successful strategy for surgical treatment of Boerhaave’s syndrome

This retrospective single-institution study presents a successful treatment strategy for Boerhaave's syndrome. During 1995-2008, 15 patients with spontaneous esophageal perforation were treated. Patients were grouped according to time from symptoms to referral (early, <24 h; late, >24 h). In group I (early, n = 8 patients) treatment comprised primary surgical esophageal repair in seven cases and endoscopic clipping in one case. In group II (late, n = 7 patients) treatment comprised esophagectomy without primary reconstruction (4 cases) or controlled esophagocutaneous fistula (3 cases). Measures of outcome included age (years), delay to diagnosis (h), severe sepsis on admission, mortality, and hospital and intensive care unit (ICU) stay. The overall hospital mortality rate was 6.6% (1/15), being 0% (0/8) in group I and 14.2% (1/7) in group II. Patient age (49.6 vs. 68.6 years, P < 0.0001), delay to diagnosis (17.75 vs. 69 h, P < 0.0001), severe sepsis on admission (0 vs. 4, P = 0.0256), and ICU stay (4 vs. 14 days, P = 0.006) were all greater in group II. Early diagnosis and carefully selected therapeutic tactics can reduce the mortality rate of Boerhaave's syndrome to an acceptably low level. Methods of organ preservation and minimally invasive techniques can be applied successfully in the treatment.

Ultrasound and MRI Prenatal Diagnosis of Esophageal Atresia: Effect on Management

Ultrasound and MRI Prenatal Diagnosis of Esophageal Atresia: Effect on Management

A Wristwatch in the Esophagus Associated with Esophageal Perforation: Report of an Intriguing Case

Foreign bodies in the esophagus are commonly seen in emergency medicine. We report here on a very rare case of a working wristwatch in the esophagus, which was successfully extracted by surgical intervention along with primary repair of the perforated cervical esophagus through a transcervical incision. This watch was impacted for 4 days in the cervical esophagus of an adult schizophrenic patient and resulted in cervical esophageal perforation associated with acute deep neck infection.

Thoracoscopic Primary Esophageal Repair in Patients With Boerhaave's Syndrome

Early diagnosis and appropriate treatment are important for a good outcome in Boerhaave's syndrome. The results of recent studies suggest that primary esophageal repair should be performed for perforations, and some authors suggest that there are benefits from thoracoscopic surgery in cases that are diagnosed early. From December 2004 to May 2010, 15 patients with Boerhaave's syndrome presented to our department; the medical records were reviewed retrospectively for preoperative signs and symptoms, interval between perforation and surgery, surgical methods, and outcomes of treatment. The patients were divided into two groups according to the surgical approach (thoracoscopy versus thoracotomy) to evaluate the outcomes of thoracoscopic surgery in patients with Boerhaave's syndrome. All patients were men, with a mean age of 53.1 years, and all underwent primary esophageal repair. Seven patients underwent a thoracoscopic approach (group A) and eight patients had a thoracotomy (group B). The mean interval between perforation and surgery was 43.5 hours (group A) and 40.2 hours (group B) (p=0.487). The mean operative time was 3.7 hours (group A) and 5.3 hours (group B) (p=0.005). Postoperative leaks were confirmed by esophagography in one patient in group A and in two patients in group B. There was no mortality in group A and one death postoperatively in group B. The results of this study suggest that thoracoscopic esophageal repair may be a good surgical alternative in patients with Boerhaave's syndrome who have a relatively stable vital sign or mild inflammation, regardless of the time interval between perforation and surgery.

Long-gap esophageal atresia treated by growth induction: the biological potential and early follow-up results

This study had two purposes. The first was to determine whether the growth procedure would allow true primary repairs of the most severe end of the esophageal atresia (EA) spectrum with the longest gaps (LG) and most rudimentary lower esophageal segments. The second goal was to provide the first short- to mid-term (3-12 years) follow-up data on the esophageal function and quality of life (QOL) data on the patients in this series. From our series of 60 LG-EA patients who underwent a growth procedure, 42 had the true primary esophageal repair completed 3 years ago. Among these, 18 had gaps over 6 cm, and for 6, only a rudimentary lower esophagus existed well below the diaphragm. No patient was turned down and all had primary repairs. These results suggest that even the most rudimentary segment has the potential to achieve normal size and that the full EA spectrum can have a primary repair. Our follow-up studies indicated that the esophageal function of these previously grown segments was very good. All contacted (40) were eating normally with only 3 receiving supplemental g-tube feeds because of other significant defects. We have actively treated significant reflux and 41/42 had fundoplication. By endoscopy (N = 15) no esophagitis was visible, but on biopsy, mild inflammation was found in 3. No conditions were found which would suggest that there would be a late deterioration or adverse consequences would arise. Based on these ongoing evaluations, the outlook seems very favorable for a good long-term QOL.

Spontaneous rupture of the oesophagus

The aim of this study was to evaluate the diagnosis, management and outcome of patients with spontaneous rupture of the oesophagus in a single centre. Between October 1993 and May 2007, 51 consecutive patients with spontaneous oesophageal rupture were evaluated with contrast radiology and flexible endoscopy. Patients with limited contamination who fulfilled specific criteria were managed by a non-operative approach, whereas the remainder underwent thoracotomy. The median time to diagnosis was 24 (range 4-604) h. Initial diagnosis was by contrast swallow in 18 of 24 patients, computed tomography in 15 of 17 and endoscopy in 18 of 18. There were no deaths among 17 patients who were managed non-operatively with targeted drainage, intravenous antimicrobials, nasogastric decompression and enteral nutrition. Of 31 patients who underwent primary thoracotomy and oesophageal repair (over a Ttube in 29), 11 died in hospital. Three patients could not be resuscitated adequately and did not have surgical intervention. Spontaneous oesophageal rupture represents a spectrum of disease. Accurate radiological and endoscopic evaluation can identify those suitable for radical non-operative treatment and those who require thoracotomy.

Acquired tracheal diverticulum following repair of tracheo-oesophageal fistula: Endoscopic management

Remnant tracheal diverticulum following the repair of a tracheo-oesophageal fistula (TOF) is an uncommon complication. The majority of lesions are discovered and repaired at the time of primary oesophageal repair. Respiratory complications are reported to be a major cause for morbidity after surgical repair of a TOF. They include anastomotic stricture, oesophageal dysmotility and tracheomalacia. These case reports highlight the need to be wary of tracheal diverticulum as a complication following the repair of a TOF, which may cause difficulty in intubation, airway compromise or an inability to provide effective ventilation. An effective endoscopic surgical technique has been described.

Primary esophageal repair in Boerhaave's syndrome

Boerhaave's syndrome is the most severe disease in the esophageal perforation. The purpose of this report is to evaluate the outcome in patients who were treated with primary repair for Boerhaave's syndrome regardless of the time interval. From 1997 to July 2007, 10 patients with Boerhaave's syndrome were treated with primary repair regardless of the time interval. The interval between rupture and initial treatment was less than 24 hours in five patients (50.0%) and more than 24 hours in the other five patients (50.0%). There was no operative mortality and five postoperative leaks. Of these five patients with postoperative leaks, one received primary repair for less than 24 hours (20%) and four received operation for more than 24 hours (80%). However, postoperative leaks were managed by non-operative methods and resolved within 2 weeks. The time interval between perforation and operative intervention should not prejudice the surgeon against primary repair of Boerhaave's syndrome. Although a high incidence of postoperative leak occurred in patients who were operated on for more than 24 hours, its management is not hard to perform and its prognosis was not poor.

Early “sham” feeding of neonates promotes oral feeding after delayed primary repair of major congenital esophageal anomalies

Background To promote the appropriate and early development of the suck and swallow mechanism, we instituted a “sham” feeding protocol in infants undergoing delayed primary repair of an esophageal atresia anomaly. Methods Four patients who were born with esophageal atresia required delayed repair and were sham fed before surgical correction. Each infant started with a small volume of oral feeds by using a bottle/nipple with a continuous Replogle suction system placed nasally into the esophageal pouch. They were carefully monitored. The volume of feeds was slowly increased as tolerated. Results All infants successfully completed the sham feeding protocol before undergoing delayed primary esophageal repair. After repair, they had a shortened time to full oral feeding. Conclusion Our “sham” feeding protocol is safe and very effective in early development of oral feeding mechanisms and shortens time to complete oral feeding after delayed esophageal repair.

Cervical cellulitis and mediastinitis following esophageal perforation: A case report

Chicken bone is one of the most frequent foreign bodies (FB) associated with upper esophageal perforation. Upper digestive tract penetrating FB may lead to life threatening complications and requires prompt management. We present the case of a 52-year-old man who sustained an upper esophageal perforation associated with cervical cellulitis and mediastinitis. Following CT-scan evidence of FB penetrating the esophagus, the impacted FB was successfully extracted under rigid esophagoscopy. Direct suture was required to close the esophageal perforation. Cervical and mediastinal drainage were made immediately. Naso-gastric tube decompression, broad-spectrum intravenous antibiotics, and parenteral hyperalimentation were administered for 10 d postoperatively. An esophagogram at d 10 revealed no leak at the repair site, and oral alimentation was successfully reinstituted. Rigid endoscope management of FB esophageal penetration is a simple, safe and effective procedure. Primary esophageal repair with drainage of all affected compartments are necessary to avoid life-threatening complications.

Congenital esophageal stenosis associated with esophageal atresia: new concepts

Congenital esophageal stenosis (CES) is suspected by a fixed intrinsic narrowing of the esophagus that affects the normal swallowing mechanism. The diagnosis is only confirmed by histopathologic picture, which may show fibromuscular disease (FMD) or tracheobronchial remnants (TBR). The latter involves ciliated pseudo stratified columnar epithelium, seromucous glands or cartilage each alone or in combination. The aim of this study is to document the usefulness of histologic picture of surgical specimens obtained from the lower esophageal pouch (LEP) during primary repair in detecting cases of CES associated with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF). Over an 8-year period, 57 consecutive cases operated upon for EA with or without TEF were subjected for histologic examination of surgical specimens obtained from the tip of the LEP. Cases that histologically showed FMD or TBR were included. The usefulness of this histologic picture as a diagnostic and therapeutic aid is assessed. Methods of treatment and outcome were also reviewed. Eight patients out of 57 (14%) had a histologic picture suggestive of CES, two with FMD, four with TBR without cartilage and two with cartilage. Out of 57 patients, 23 developed strictures, six of them had positive biopsies suggestive of CES. One patient with TBR without cartilage did not have stricture. Another case of pure atresia had LEP resection and gastric pull up showed cartilage involving the whole lower esophagus. Excluding the case of pure EA with gastric pull up, all patients suffered from feeding problems and recurrent aspiration. Fluoroscopic barium studies showed late onset minor dysmotility in five patients and late onset major dysmotility in two. All cases studied showed significant gastro-esophageal reflux (GER). Stricture was seen at the anastomotic site extending distally in the two fibromuscular cases and one case with cartilage, at the anastomotic site in three cases with TBR without cartilage. Anti reflux surgical procedures were performed in four patients without benefit in two patients with major dysmotility. Dilatation was successful in the three patients with TBR without cartilage. One patient with cartilage had resection of the anastomotic site and required frequent dilatations and is now doing well. A case of FMD did not improve after frequent dilatations and myotomy together with Nissen's fundoplication and required resection while the other case of FMD responded partially to dilatations. Cartilage in cases of CES requires surgical resection. Those with TBR without cartilage may not develop stricture. If stricture develops, it responds well to dilatation and patients have good clinical outcomes. Unlike isolated CES, GER is a significant feature in CES with EA. Anti reflux procedures should be avoided before definitive surgery for the stricture and if necessary a partial wrap with gastrostomy is recommended. CES should be considered in the etiology of anastomotic stricture. Taking a surgical specimen routinely from the tip of the LEP during primary esophageal repair for histologic studies is highly recommended.

Oesophageal perforation: primary suture repair reinforced with absorbable mesh and fibrin glue.

Abstract Despite major advances in the management of intrathoracic oesophageal perforation, morbidity and mortality rates remain high1,2. Currently accepted management3 includes early thoracotomy, cleansing of the mediastinum, oesophageal suture and pleural space drainage. Even when performed at an early stage, primary oesophageal suture repair remains precarious, however. Experience of oesophageal primary suture reinforced with absorbable mesh and fibrin glue is reported.

Success and failure with neonatal tracheo-oesophageal anomalies

In seven and a half years, one surgical team treated 67 consecutive neonates with oesophageal atresia and/or tracheo-oesophageal fistula. According to Waterston's classification, 28 were in group A, 12 in group B and 27 in group C. The mortality rate during the initial admission was 10 per cent, all seven deaths being unavoidable in infants in group C with multiple anomalies. Birthweight alone had no bearing upon the chances of survival. Primary oesophageal repair, including one suture-fistula procedure and one delayed primary repair, was attempted in 54 (84 per cent) of the 64 patients with atresia and was successful in 46 (85 per cent). All three H-type tracheo-oesophageal fistulae were successfully divided in infants in group A. Recurrent tracheo-oesophageal fistula developed in four (7 per cent) infants, one of whom (group A) underwent successful repair. One disrupted anastomosis was successfully resutured (group A), so an intact oesophagus was finally achieved in 51 patients, of whom six (12 per cent) developed anastomotic strictures and 21 (41 per cent) underwent surgery for gastro-oesophageal reflux. Of the 60 early survivors, 10 (17 per cent) underwent aortopexy for tracheomalacia. Whenever possible, primary repair is advocated in all infants. Even for those in group C with multiple, severe associated anomalies, the combined early and late mortality was no greater following primary repair (7 died of 12 operated) than after staged repair (4 died of 7 operated), but major anastomotic complications were more common in infants in group C (5 out of 19) than in those in groups A and B (3 out of 38).