Can 'long-gap' esophageal atresia be safely managed at home while awaiting anastomosis?

Background: Neonates with [ldquo ]long gap[rdquo ] esophageal atresia (EA) are often managed with gastrostomy and tube drainage of the proximal pouch for a number of months while awaiting definitive repair. Because of the risk of aspiration and need for complex nursing care, most remain hospitalized during this time. However, prolonged hospitalization utilizes scarce resources and may be difficult for many families. Methods: The authors report on 5 patients who were treated successfully at home while awaiting esophageal anastomosis. Results: Four patients had pure EA (one had a duodenal atresia), and one had a distal fistula. Gestational ages ranged from 31 to 41 weeks. All had a gastrostomy within days of birth. Age at definitive repair ranged from 6 to 12 months. Time at home while awaiting anastomosis ranged from 42 to 113 days. Care at home included nursing care, suction equipment and training, gastrostomy feeding, and ability to perform cardiopulmonary resuscitation. The only complications noted while at home consisted of ear infection in one patient and recurrent upper respiratory tract infections in another patient. Conclusions: Selected patients with long gap esophageal atresia can be treated safely at home while awaiting esophageal anastomosis. Success of this approach depends on a motivated, reliable family, and adequate support from community health care providers. J Pediatr Surg 38:705-708. [copy ] 2003 Elsevier Inc. All rights reserved.     

Endoscopic follow-up in esophageal atresia-for how long is it necessary?

Purpose: The aim of this study was to find out how long it is necessary to follow-up esophageal atresia patients endoscopically to prevent irreversible mucosal changes. Methods: Seventy-four of 79 long-term survivors with esophageal atresia and primary anastomosis underwent a total of 322 esophagogastroduodenoscopies during a follow-up period of 0.5 to 19 years (mean, 10.3) after the primary operation. For analysis, the biopsy findings were divided into 2 groups: good, histologically normal or only mildly inflamed mucosa; unfavorable, moderate or severe esophagitis or gastric metaplasia. Fundoplication irrespective of indications was considered unfavorable. The results were analyzed using actuarial survival analysis; the changing point was when a [ldquo ]good[rdquo ] turned into [ldquo ]unfavorable.[rdquo ] Results: Forty-five patients (61%) remained in the [ldquo ]good[rdquo ] group throughout the study period; 15 of those (20%) had completely normal findings. Nine patients (12%) had moderate, one (1%) had severe esophagitis, and 13 patients (18%) had gastric metaplasia. Fundoplication was performed on 21 patients (28%). Conclusions: About 40% of esophageal atresia patients eventually have significant esophageal mucosal pathology or need to have a fundoplication. The majority of these changes appear before the age of 3 years. Routine endoscopic follow-up of esophageal atresia patients is recommended at least to the age of 3 years. J Pediatr Surg 38:702-704. [copy ] 2003 Elsevier Inc. All rights reserved.     

Application of collagen sponge scaffold to muscular defects of the esophagus: an experimental study in piglets

Background/Purpose: Circular myotomy often has been used to elongate the esophagus in end-to-end anastomosis for esophageal atresia with a long gap. Defects of the muscular layer often have led to the ballooning of the esophagus during the postoperative course. Recently, successful regeneration of the neo-esophagus using collagen sponge scaffold (CSS) for self-tissue regeneration has been reported. The authors applied this CSS to the muscular defect after circular myotomy in animal experiments. Methods: Myectomy or myotomy of the cervical or thoracic esophagus was performed in 11 piglets. CSS was applied to the amuscular site, which was 20 to 30 mm in length, to examine the regeneration of the muscular layer in 8 piglets (CSS group). The amuscular site was not wrapped by CSS in 3 piglets as a control (control group). Two months after operation, the animals were killed, and their operated esophagi were examined macroscopically and microscopically. Results: All 11 piglets increased in weight normally without any sign of dysphagia or vomiting. The amuscular site onto which CSS was implanted was covered with loose connective tissue, but muscle regeneration was not found microscopically. The amuscular site, which had been wrapped by CSS, was not distended in any of the 8 piglets when inflated, and ballooning was observed in all 3 of the control group. Conclusion: The results suggested that application of CSS to the site of circular myotomy for esophageal atresia with a long gap might not induce muscle regeneration but could prevent postoperative ballooning by inducing connective tissue growth. J Pediatr Surg 37:1409-1413.     

Quality of life after gastric transposition for oesophageal atresia

Background/Purpose: A small proportion of infants born with oesophageal atresia in which the gap between the 2 ends of the oesophagus is too great for an end-to-end anastomosis will require oesophageal replacement. Since 1981 the author's procedure of choice for oesophageal replacement has been gastric transposition. The long-term functional outcome appears to be satisfactory, but the quality of life of these patients has not been investigated formally. This report assesses the health-related quality of life (QOL) of 2 groups of patients born with oesophageal atresia who have undergone gastric transposition. Methods: The study group comprised 28 patients aged 2 to 22 years who resided in England. Group 1 (n = 13), comprised patients who had undergone cervical oesophagostomy and gastrostomy without attempt at oesophageal anastomosis; group 2 (n = 15), comprised patients who had undergone previous attempts at reconstruction or replacement. QOL was assessed using modified versions of the Gastrointestinal Quality Of Life Index (GIQLI). Results: QOL scores based on patients' responses showed no significant differences between the groups (124 v 119). However, the disease-specific symptom scores showed that patients in group 1 experienced fewer symptoms compared with those in group 2. Additionally, based on parental responses, patients in group 1 had higher QOL scores than those in group 2. QOL scores for patients aged 2 to 4 years (n = 5) did not differ between the groups (81 v 92, not significant). Conclusions: The quality of life for patients with oesophageal atresia undergoing gastric transposition was generally unimpaired by any side effects of gastric transposition. Patients undergoing gastric transposition as a primary procedure experienced fewer disease-specific symptoms in the medium term compared with patients who had undergone previous unsuccessful attempts at reconstruction or replacement of their oesophagus. J Pediatr Surg 38:53-57.     

An exclusively intraabdominal distal esophageal segment prevents primary delayed anastomosis in children with pure esophageal atresia

Purpose: The aim of this study was to evaluate the outcome of children with pure esophageal atresia (EA) managed by the strategy of delayed primary anastomosis and to find parameters to identify which of these children will have satisfactory esophageal growth to permit a successful primary esophageal anastomosis. Methods: Sixteen children with pure EA were treated by delayed anastomosis strategy from June 1992 to May 2001 at the Instituto da Crian[ccedil]a-University of S[atilde]o Paulo Medical School. The treatment consisted of creating an early feeding gastrostomy, continuous upper pouch suction, and periodic radiologic/endoscopic assessments of the gap between the superior and inferior esophageal pouches. The first assessment was done 4 weeks after the initial gastrostomy. Subsequent assessments were done, when necessary, 4 to 10 weeks after the first one. Children with the gap between the pouches corresponding to 2 vertebral bodies or less were considered for primary repair. Results: The waiting period ranged from 8 to 14 weeks. Five children were considered for primary repair (31.3%). All of them had an end-to-end esophageal anastomosis performed without tension. Eleven children (68.7%) did not show significant esophageal growth after the waiting period and were not considered for primary esophageal anastomosis. Eight of them (50%) showed an entirely intraabdominal distal pouch at the first assessment. Conclusions: (1) Infants with an exclusively intraabdominal distal pouch at the first evaluation will not reach sufficient elongation of the pouches and should be considered early as a candidate for esophageal replacement. (2) Combined radiologic/endoscopic assessment and the measurement of the gap in terms of vertebral bodies is a practical and reliable method to predict the feasibility of primary anastomosis. (3) Using these criteria, primary anastomosis could be accomplished in less than a third of cases.     

Thoracoscopic repair of tracheoesophageal fistula in newborns

Background: Advancements in minimally invasive surgery in neonates have allowed even the most complex neonatal procedures to be approached using these techniques. Methods: During a period of 15 months, 8 patients born with a proximal esophageal atresia and a distal tracheoesophageal fistula underwent repair thoracoscopically. Weights ranged from 2.1 to 3.4 kg and operating times ranged from 55 to 120 minutes.Results: All procedures were completed successfully thoracoscopically, and there were no operative complications. One patient had a small leak on day 4 that resolved spontaneously on day 8. All other patients were shown to have a patent anastomosis with no leak by Barium swallow on day 5. Conclusion: This initial report shows that esophageal repair in the neonate is technically feasible and may provide advantages in terms of exposure and esophageal length, as well as the recognized advantages of avoiding a thoracotomy. J Pediatr Surg 37:869-872.     

Fundal tube esophagoplasty for esophageal reconstruction in atresia

Purpose: This is a report of 4 patients with long gap/pure esophageal atresia, who, after an initial gastrostomy and cervical esophagostomy at birth, were treated with a new technique called fundal tube esophagoplasty.Methods: The technique consisted of mobilization of the distal esophageal stump, division of the left gastric artery, and mobilization of upper half of stomach. Both walls of the stomach were incised in the region of the fundus starting on the lesser curvature in such a way that a tube was created out of the fundus, and the native esophageal stump appears to be an extension of this neoesophagus. This neoesophagus was brought to the neck by the retrosternal route and in a second stage, esophago-esophageal anastomosis was made. Thal’s fundoplication was added in 2 cases.Results: All the stages have been completed successfully in 4 babies. They have been followed up for periods ranging from 8 to 24 months after restoration of esophageal continuity. All the children are thriving.Conclusions: Fundal tube esophagoplasty (1) serves to provide a neoesophagus of natural caliber, (2) utilizes native esophagus in reconstruction, (3) does not produce a space-occupying problem in the mediastinum, and (4) preserves the gastric reservoir function. In this early experience, it appears to fulfill all the requirements of a good esophageal substitute.     

Robotic-assisted esophagoesophagostomy

Background/Purpose: Minimally invasive repair of esophageal atresia has been described but remains technically challenging. Robotic surgical systems address many of these technical challenges. The purpose of this study was to develop the procedure for and evaluate the technical feasibility of performing a robotic-assisted esophagoesophagostomy using the Zeus Robotic Surgical System. Methods: Esophagoesophagostomy was performed in 10 piglets using thoracoscopic (control, n = 5) and robotic-assisted (Zeus, experimental, n = 5) approaches. An interrupted esophageal anastomosis using intracorporeal knot tying techniques was performed and evaluated for leak, narrowing, caliber, and mucosal approximation. Anesthesia, operative, anastomotic, and robotic set-up times were recorded as was the number of stitches used. Results: All 10 anastomoses were patent with no narrowing and with excellent mucosal approximation. One anastomosis in the control group had a small leak. There was no statistically significant difference between the groups for the parameters measured. Weight (kg): control (C), 6.4 [plusmn] 0.8; experimental (E), 6.3 [plusmn] 1.0, P = .08. Times (min): anesthesia, C-124 [plusmn] 25, E-151 [plusmn] 20, P = .09; operative, C-97 [plusmn] 21, E-131 [plusmn] 27, P = .06; anastomotic, C-89 [plusmn] 20, E-125 [plusmn] 34, P = .08; robotic set-up, C-6.4 [plusmn] 9.3, E-15.6 [plusmn] 20, P = 0.13. Stitches (No.): C-11.8 [plusmn] 0.8, E-12.0 [plusmn] 1.2, P = .7. Caliber (French):C-18F-5; E-18F-4, 14F-1. Conclusion: Robotic-assisted esophagoesophagostomy is technically feasible and offers an alternative approach to thoracoscopic repair of esophageal atresia. J Pediatr Surg 37:983-985.     

Feasibility of thoracoscopic repair of esophageal atresia with distal fistula

Backgound/Purpose: Evaluation of the feasibility of thoracoscopic correction of esophageal atresia with distal fistula. Methods: Eight consecutive neonates with esophageal atresia and distal fistula were treated thoracoscopically. Mean birth weight was 3,048 g (range, 2,140 to 3,770). The patients were intubated endotracheally and placed in a [frac34] left prone position. Three cannulae were inserted along the inferior tip of the scapula. CO₂ was insufflated at a pressure of 5mm Hg and a flow of 0.5 L/min. The fistula was either clipped or ligated. The proximal esophagus was opened and an anastomosis was made over a 6F or 8F nasogastric tube with interrupted 5-0 Vicryl. Results: All procedures were completed thoracoscopically without major peroperative complications. The mean operating time was 198 minutes (range, 138 to 250). One patient had a major leak, resulting in a stormy postoperative course, but the leak healed on conservative treatment. This patient and 3 others had stenosis requiring dilatation, respectively, 3, 6, 12, and 1 times. The babies were fed after a median period of 8 days. The median hospital stay was 13 days. Conclusions: Thoracoscopic repair of esophageal atresia with distal fistula is feasible. Larger series are needed to determine the exact place of the thoracoscopic approach.     

Long gap esophageal atresia: lengthening technique and primary anastomosis

Objective

The treatment of long gap esophageal atresia remains a major surgical challenge. The authors describe a modification of a lengthening technique based on tissue expansion to avoid sutures cutting through the esophagus.

Methods

Between January 2004 and August 2006, 4 patients did not respond to stretching, and underwent this modified esophageal lengthening technique using silastic tubes.

Results and Follow-up

All infants recovered and have an intact esophagus. All infants developed gastroesophageal reflux. Thal antireflux procedure was performed in the first infant. The other 3 patients were managed conservatively. Follow-up ranged between 6 and 34 months.

Conclusions

The tissue expansion principle can be successfully applied in the esophagus through external traction. Silastic tube fixation at esophageal ends may help to apply even traction and avoid sutures cutting through the esophageal tissue.     

Slide cricotracheoplasty: a novel surgical technique for congenital cricotracheal stenosis

Purpose: The aim of this study was to introduce a new surgical technique for the correction of congenital cricotracheal stenosis. Methods: A 5-day-old girl presented with esophageal atresia and congenital cricotracheal stenosis. After successfully correcting her esophageal atresia, the authors chose to use a type of slide cricotracheoplasty, which was a modification of slide tracheoplasty and anterior cricoid split. Results: The postoperative period was remarkably uneventful except for minor subcutaneous emphysema, and the midterm results were excellent. Conclusions: Slide cricotracheoplasty produced a good result and offered the same advantages as slide tracheoplasty. The authors believe that the described technique offers an efficient surgical procedure for the single-staged correction of congenital cricotracheal stenosis.     

Magnetic resonance cholangiography for the diagnosis of biliary atresia

Purpose: The aim of this study was to evaluate the usefulness of magnetic resonance cholangiography (MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods: Forty-seven consecutive infants with cholestatic jaundice underwent single-shot MRC. The diagnosis of biliary atresia was made by MRC based on the nonvisualization of extrahepatic bile ducts and excluded on the basis of the complete visualization of extrahepatic bile ducts. The final diagnosis of biliary atresia (BA group, n = 23) or nonbiliary atresia (NBA group, n = 24) was established by operation or clinical follow-up until the jaundice resolved. Results: The extrahepatic bile ducts including the gallbladder, the cystic duct, the common bile duct, and the common hepatic duct were visualized in 23 of the 24 infants of the NBA group. The extrahepatic bile ducts, except the gallbladder, were not depicted in any infant of the BA group. MRC had an accuracy of 98%, sensitivity of 100% and specificity of 96%, for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. Conclusions: MRC is a very reliable noninvasive imaging modality for the diagnosis of biliary atresia. In infants with cholestatic jaundice and considered for exploratory laparotomy, MRC is recommended to avoid unnecessary surgery.     

Endoscopic balloon dilatation of esophageal strictures in infants and children: 17 years' experience and a literature review

Purpose: Whereas endoscopic balloon dilatation (EBD) of benign esophageal strictures is an established mode of therapy in adults, this has not been accepted universally in the pediatric population. The aim of this study is to report the safety, efficacy, and long-term results of EBD for children in the authors’ center.Methods: Between 1986 and 2002, a total of 77 children (median age, 1.8 years; range, 2 months to 20 years) were treated by EBD for various causes: 2 had achalasia, and 75 had esophageal strictures (postesophageal atresia repair, 63; reflux esophagitis, 7; postfundoplication, 2; caustic injury, 3). Dilatations were performed using flexible endoscopy and fluoroscopic screening under general anesthesia.Results: A total of 260 dilatations were carried out with the mean number of EBD per patient being 3.4 (range, 1 to 19). A mean period of 5 months (maximum, 28 months) for each patient was required. Four complications of esophageal perforations (1.5%) were observed, but only one required surgical repair because of persistent leakage. The remaining patients have undergone long-term follow-up (median follow-up, 6.6 years), and all are asymptomatic.Conclusions: This large series has shown that EBD can provide a safe and effective mean of relieving esophageal strictures with good long-term results.     

High-dose steroids,ursodeoxycholic acid,and chronic intravenous antibiotics improve bile flow after Kasai procedure in infants with biliary atresia

Background/Purpose: Early reports suggest that the use of steroids after Kasai portoenterostomy may improve bile flow and outcome in infants with biliary atresia. Methods: Of 28 infants with biliary atresia, half received adjuvant high-dose steroids, and half received standard therapy. Infants in the steroid group (n = 14) received intravenous solumedrol (taper of 10, 8, 6, 5, 4, 3, 2 mg/kg/d), followed by 8 to 12 weeks of prednisone (2 mg/kg/d). The steroid protocol also included ursodeoxycholic acid indefinitely and intravenous antibiotics for 8 to 12 weeks followed by oral antibiotic prophylaxis. Infants in the standard therapy group (n = 14) received no steroids, occasional ursodeoxycholic acid, and perioperative intravenous antibiotics followed by oral antibiotic prophylaxis. The infants were not assigned randomly, but rather received standard therapy or adjuvant steroid therapy according to individual surgeon preference. Results: Eleven of 14 (79%) in the steroid group and 3 of 14 (21%) in the standard therapy group had a conjugated bilirubin level less than 1.0 within 3 to 4 months of surgery (P [lt ] .001). Fewer patients in the steroid group (21% v 85%) required liver transplantation or died during the first year of life (P [lt ] .001). Infants in the steroid group did better despite the fact that this group included 5 infants with biliary atresia-polysplenia-heterotaxia syndrome, a subgroup that might have been expected to have a poor prognosis. Neither bile duct size nor liver histology was a reliable predictor of success or failure in either group. Conclusions: Adjuvant therapy using high-dose steroids, ursodeoxycholic acid, and intravenous antibiotics may accelerate the clearance of jaundice and decrease the need for early liver transplantation after Kasai portoenterostomy. J Pediatr Surg 38:406-411.     

Application of the principles of myotomy and strictureplasty for treatment of esophageal strictures

Background/Purpose: Strictures of the esophagus in the pediatric population may be congenital or acquired. Regardless of the etiology, the treatment goal is to relieve the symptoms and allow patients to eat normally. The cornerstone of nonoperative management is repeated esophageal dilatations. However, when nonoperative management fails, operative intervention becomes necessary. In this report the authors present 4 cases of severe upper esophageal strictures managed by applying the principles of myotomy and strictureplasty. Methods: Retrospective review of 4 patients undergoing esophageal stricturotomy from January 1, 1993 to January 1, 2000 was conducted at Childrens Hospital Los Angeles, with a mean follow-up period of 5 years. Results: Three of the 4 patients reported in this report are doing well and have not required any further surgical intervention. One patient in whom additional strictures developed has had a microvascularized free jejunal graft and also is doing well. Conclusions: Currently accepted surgical management of strictures includes resection of the affected segment with end-to-end anastomosis or esophageal replacement. The authors propose that before embarking on a long and technically hazardous operation, consideration should be given to stricturotomy. In cases of isolated strictures, this may be the preferred approach.     

Successful two-stage thoracoscopic repair of long-gap esophageal atresia using simple internal traction and delayed primary anastomosis in a neonate: report of a case

The optimal method of treating long-gap esophageal atresia has not been established; however, the native esophagus is recognized as one of the best materials to use for bridging a long gap. Several case reports describe long-gap esophageal atresia being treated successfully via thoracotomy, by applying external traction sutures to the proximal and distal ends of the esophagus, thereby gradually elongating the esophagus prior to delayed primary anastomosis. However, this method carries a risk of infection and disruption of the esophageal ends. We devised a simple method of internal traction for esophageal elongation, which we performed as a two-step thoracoscopic procedure to treat a neonate with long-gap esophageal atresia.     

Thoracoscopic elongation of the esophagus in long gap esophageal atresia

Long gap esophageal atresia in which a primary anastomosis cannot be achieved remains a challenge. Elongation of the esophagus by traction on the 2 ends has been previously described. With the advent of thoracoscopic repair of esophageal atresia, there have thus far been no reports of thoracoscopic repair of long gap esophageal atresia. This paper describes the first successful repair of long gap esophageal atresia by thoracoscopic traction of the 2 esophageal ends and delayed thoracoscopic anastomosis.     

Outcomes in esophageal atresia and tracheoesophageal fistula

Background/Purpose: The purpose of this analysis was to investigate outcomes in newborns with esophageal atresia (EA) or tracheoesophageal fistula (TEF) with respect to prognostic classifications and complications.Methods: Charts of all 144 infants with EA/TEF treated at British Columbia Children’s Hospital (BCCH) from 1984 to 2000 were reviewed. Patient demographics, frequency of associated anomalies, and details of management and outcomes were examined.Results: Applying the Waterston prognostic classification to our patient population, survival rate was 100% for class A, 100% for class B, and 80% for class C. The Montreal classification survival rate was 92% for class I and 71% for class II (P = .08). Using the Spitz classification, survival rate was 99% for type I, 84% for type II, and 43% for type III (P < .05). The Bremen classification survival rate was 95% “without complications” and 71% “with complications.” Complications included stricture (52%), gastroesophageal reflux (31%), anastomotic leakage (8%), recurrent fistula (8%), and pneumonia (6%). Seventeen patients underwent fundoplication for gastroesophageal reflux, 16 pre-1992 and one post-1992.Conclusions: Comparing the major prognostic classifications, the Spitz classification scheme was found to be most applicable. In our institution, the trend in management of gastroesophageal reflux after repair of EA/TEF has moved away from fundoplication toward medical management.     

Urinary sulfated bile acids: a new simple urine test for cholestasis in infants and children

Background/Purpose: Measurement of urinary sulfated bile acid (USBA) has been reported as a simple urine test that reflects the degree of cholestasis. The authors report the diagnostic value of this new laboratory test in various cholestatic conditions affecting infants and children. Methods: A urine sample was collected from 4 surgical neonates with parenteral nutrition[ndash ]induced cholestasis and 48 patients with biliary atresia (BA). USBA was measured by direct enzymatic assay. Results: In 3 patients receiving parenteral nutrition, USBA increased with caloric gains. For one surgical patient, a decrease in calories because of liver dysfunction resulted in a decrease of USBA, closely reflecting the fluctuations of caloric intake. In patients with BA, a significant positive correlation was noted between serum bile acid and USBA (r = 0.85; P [lt ] .01). Ten of 14 febrile episodes in 6 patients with liver dysfunction and increased C-reactive protein showed elevated USBA, thus diagnosed as cholangitis. Four febrile episodes caused by viral infection showed no elevation of USBA. Conclusions: USBA is a simple and sensitive noninvasive test reflecting the degree of cholestasis in infants and children. USBA correlated highly with serum bile acid levels and may be helpful in diagnosis of cholestasis caused by cholangitis without blood sampling.