Congenital esophageal stenosis: Therapeutic strategy based on etiology

Background/Purpose: The diagnosis and treatment of congenital esophageal stenosis (CES) can be a vexing clinical problem. This study aims at determining the ideal therapeutic strategy for the management of CES. Methods: Medical records of patients with CES were reviewed retrospectively with regard to diagnostic method, therapy, and outcome. Results: During the last 29 years, 36 patients, aged 1 month to 9 years, were found to have CES. Diagnosis was made by fluoroscopy, esophagoscopy, endoscopic ultrasonography (EUS), and pathologic examination. Of the 36 patients, 15 had tracheobronchial remnants (TBR), 13 had fibromuscular stenosis (FMS), 5 had membranous stenosis (MS), and 3 had multiple stenoses (MPS). EUS was diagnostic in distinguishing TBR from FMS. All patients with TBR underwent operative repair in which 13 patients had resection and anastomosis with or without preoperative dilation. The 14th patient had enucleation and the 15th underwent myotomy. Postoperative dilation was needed 2.2 times for 2.1 months on average in this group of patients. Ten patients with FMS were treated by dilation only (a mean of 4.7 dilations was required for a duration of 2.6 years), whereas 3 patients underwent open surgical intervention. Most patients with MS or MPS were treated by dilation (with or without endoscopic resection of the membranes), whereas 2 patients had surgical intervention. All patients in this study are doing well after a mean follow-up period of 5.3 years (range, 0.5 to 25 years) without any further operative interventions. Conclusions: If the etiologic diagnosis of CES is unclear, EUS is useful in distinguishing TBR from FMS. This distinction is critical, because patients with TBR should undergo surgical resection, whereas most cases of FMS, MS, and MPS can be treated with esophageal dilation alone.     

Endoscopic management of congenital esophageal stenosis

Background/Purpose

Congenital esophageal stenosis (CES) is a rare malformation. Endoscopic dilations represent a therapeutic option. This study retrospectively evaluated the efficacy and safety of a conservative treatment of CES.

Patients and Methods

Patients diagnosed with CES since 1980 by a barium study or endoscopy were reviewed. Endoscopic ultrasonography (Olympus UM-3R-20-MHz radial miniprobe, Olympus Corporation, Tokyo, Japan), available from 2001, allowed for the differential diagnosis of tracheobronchial remnants (TBR) and fibromuscular hypertrophy (FMH) CES. All children underwent conservative treatment by endoscopic dilations (hydrostatic and Savary).

Results

Forty-seven patients (20 men) had CES. Fifteen were associated with esophageal atresia; and 8, with Down syndrome. Mean age at the diagnosis was 28.3 months (range, 1 day to 146 months). Symptoms were solid food refusal, regurgitation, vomiting, and dysphagia. Congenital esophageal stenosis was located in the distal esophagus. Endoscopic ultrasonography demonstrated TBR and FMH in 6 patients. One hundred forty-eight dilations in 47 patients were performed. The stenosis healed in 45 (95.7%). Complications were 5 (10.6%) esophageal perforations, hydrostatic (3/32, or 9.3%), and Savary (2/116, or 1.7%). At follow-up, 1 patient with FMH CES and 1 patient with TBR CES required operation for persistent dysphagia.

Conclusions

The conservative treatment yielded positive outcomes in CES. Endoscopic ultrasonography allows for a correct diagnosis of TBR/FMH CES. A surgical approach should be reserved for CES not responsive to dilations.     

Management of congenital esophageal stenosis

Background/Purpose: The authors report the incidence, diagnosis, and treatment methods of congenital esophageal stenosis (CES) at their institution. Methods: A retrospective analysis of 123 patients with tracheoesophageal anomalies in a pediatric hospital between 1980 and 1999 was performed. Charts were reviewed for patient demographics, presence of true CES, associated congenital anomalies, method of diagnosis, methods of treatment, and histopathology. Results: Six of the 123 patients (4.9%) had true CES (one patient had 2 separate stenoses). Two patients had isolated CES, one patient had CES with isolated tracheoesophageal fistula (TEF), one patient had CES with isolated esophageal atresia (EA), and 2 patients had CES with EA/TEF. Diagnoses were made with an upper gastrointestinal (GI) contrast study in 5 patients, and one patient had one of 2 stenoses diagnosed by prenatal ultrasound and the other diagnosed intraoperatively. Four of the 7 stenoses were treated with surgical resection, and the remainder was treated with esophageal dilatation. Histopathology from the 4 resected stenoses showed tracheobronchial remnants in 3 specimens and submucosal thickening in 1 specimen. Conclusions: Although isolated CES is rare (2 of 123 = 1.6%), CES associated with other tracheoesophageal anomalies has a higher incidence (4 of 123 = 3.25%). Patients with this lesion should be treated first with dilatation. If ineffective, resection is required. J Pediatr Surg 37:1024-1026.     

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.     

Lengthening technique for long gap esophageal atresia and early anastomosis

Background/Purpose: The treatment of long gap esophageal atresia remains a major surgical challenge. Whereas many approaches have been used for this problem, none are ideal. The authors used a technique originally described by Dr John E. Foker and accomplished early repair in 3 infants with long gap atresia. Methods: Three infants with esophageal atresia underwent thoracotomy shortly after birth and had a long gap preventing primary anastomosis. External traction sutures were placed on each esophageal pouch and exteriorized through the thoracic wall. The esophageal ends were approximated 1 to 2 mm daily by traction on the sutures. Anastomosis was performed when the 2 ends came together. Results: Three infants were included (31, 34, 37 weeks gestation, weights 1.38 kg, 1.9 kg, and 2.3 kg, respectively). The esophageal gaps were 3, 5, and 4.5 cm, respectively. Definitive anastomosis was performed at 14, 17, and 10 days, respectively. Two patients had anastomotic leaks that were treated conservatively. One patient had an esophageal stenosis that required dilatation. Conclusions: This technique allowed rapid esophageal lengthening in these 3 cases and led to early repair of long gap esophageal atresia, avoiding the need for a prolonged hospitalization or eventual replacement as well as long-term swallowing difficulties. J Pediatr Surg 38:737-739. [copy ] 2003 Elsevier Inc. All rights reserved.     

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.     

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.     

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.     

Does V-A ECMO increase the likelihood of chylothorax after congenital diaphragmatic hernia repair?

Background: The authors noticed a relatively large number of patients with congenital diaphragmatic hernia (CDH) repair after extracorporeal membrane oxygenation (ECMO) who had a chylothorax (CT). The data are reviewed.Methods: The charts of patients from 1990 until 2000 with CDH, treated with or without ECMO, together with the charts of patients treated with ECMO for other reasons and patients with esophageal atresia (EA) repair were reviewed. The diagnosis of CT was made if aspirated fluid appeared chylous and contained more than 90% lymphocytes or if the triglyceride level was more than 1.50 mmol/L.Results: Eighty-nine patients with CDH were analyzed. Postoperatively, 10% had a CT—21% in CDH patients with ECMO treatment and 6% in CDH patients without ECMO treatment. This difference appeared to be significant (P < .05). The presence of a patch as independent variable for the development of CT also showed significance (P < .05).Conclusions: Chylothorax presented in almost all cases as a left-sided fluid accumulation, and a patch was present in the majority of patients with CDH. Therefor, CT should be considered the result of the severity of the defect rather than the consequence of ECMO as a therapeutic modality.     

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.     

Magill forceps extraction of upper esophageal coins

Background/Purpose: This study demonstrates a minimally invasive technique for upper esophageal coin extraction. Methods: A retrospective review was conducted of 36 children who had upper esophageal coins extracted using a Magill forceps. Results: All coins were removed without complication in approximately 45 seconds (33 on the first attempt, 3 on the second attempt). Conclusions: This technique minimizes instrumentation of the esophagus and is highly successful at removing coins lodged at or immediately below the level of the cricipharyngeus muscle. J Pediatr Surg 38:227-229.     

Usefulness of endoscopic ultrasonography in the diagnosis of congenital esophageal stenosis

Background/Purpose: Endoscopic ultrasonography (EUS) is considered a potentially useful tool to investigate structural abnormalities of the esophagus in pediatric patients, as in adults. The aim of this study was to evaluate the usefulness of EUS for the diagnosis of congenital esophageal stenosis. Methods: High-frequency catheter probe EUS was performed under general anesthesia in 2 patients who had congenital esophageal stenosis. Results: A 4-year-old boy with anorectal anomaly showed tapered narrowing in the distal esophagus, which was not ameliorated with balloon dilatation. High-frequency catheter probe EUS showed hypertrophy of the muscular layer in the esophageal wall at the narrowed portion, but no images suggested the presence of tracheobronchial remnants. The histologic diagnosis of fibromuscular hypertrophy was confirmed at esophagoplasty. A 5-month-old boy with Gross C-type esophageal atresia and symptomatic gastroesophageal reflux showed tapered narrowing in the middle esophagus on esophagography. The symptoms of stenosis were not ameliorated by balloon dilatation performed 4 times. High-frequency catheter probe EUS showed hyperechoic lesions suggesting cartilage at the esophageal narrowing. The diagnosis of tracheobronchial remnants was confirmed by the finding of 2 pieces of cartilage in the specimen obtained at the time of esophageal resection. Conclusion: EUS can be applied to show structural abnormalities of the esophageal wall even in pediatric patients with congenital esophageal stenosis and is useful for planning the therapeutic strategy.     

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.     

Aggressive conservative treatment of esophageal perforations in children

Background/Purpose: In contrast with adult patients in whom surgical closure of the defect is preferred, nonoperative treatment has been the usual approach for esophageal perforation (EP) in children. This report aims to assess whether this strategy stands the passage of time. Methods: We reviewed retrospectively the charts of 17 patients aged 5.3 [plusmn] 0.9 years (mean [plusmn] SD) treated at our institution for EP between 1991 and 2001. Results: Nineteen episodes of EP were caused by stricture dilation in 9 cases, foreign body extraction in 3, and blunt trauma and sclerosis of varices in 2 cases each. The remaining child had multiple gastrointestinal perforations in the course of chemotherapy for leukemia. Vigorous treatment, consisting of nasopharyngeal aspiration, wide spectrum antibiotics, prompt drainage of effusions and either parenteral or infraesophageal nutritition, was implemented immediately after diagnosis. Perforations were closed without direct surgery in 18 of 19 episodes (16 of 17 children). One or more pleural drains were inserted in 12 cases, and pericardial drainage was required once. Seven gastrostomies, 2 jejunostomies, and one esophagostomy were performed. Several major abdominal operations were necessary to repair concomitant lesions in a child who sustained severe blunt abdominal trauma and in the patient with leukemic perforations. All patients survived, and all recovered esophageal function. However, 2 with intractable lye strictures ultimately required esophageal replacement. The only patient in whom a direct approach for esophageal necrosis due to variceal endosclerosis was unavoidable, lost her organ and had a retrosternal colonic interposition after a successful portosystemic shunt. Excluding patients with other concomitant lesions and the patient who underwent surgery, median length of stay was 11 days (range, 6 to 47). Conclusions: Prompt and aggressive nonoperative treatment of esophageal perforations in children allows survival with conservation of the organ in most cases and remains, in the authors[apos ] hands, the first therapeutic choice at this age. J Pediatr Surg 38:685-689. [copy ] 2003 Elsevier Inc. All rights reserved.     

Uptime as a measure of recovery in children postappendectomy

Purpose: The aim of this study was to objectively measure recovery time after open and laparoscopic appendectomy using an activity monitor (PAL 1).Methods: The PAL 1 records the amount of time that the subject is upright (uptime). Children wore the PAL 1 for 10 days continuously, beginning within the first 48 hours postoperatively.Results: Uptime data were collected for 5 or more days for 42 children (open, n = 16; laparoscopic, n = 26). All children had low levels of uptime in the initial postoperative period. There was weak evidence of a quicker recovery rate for children in the laparoscopic group (P = .09). The difference in mean uptime between groups was of statistical significance by day 7 postoperatively with children in the laparoscopic group having a higher mean uptime than those in the open group (difference of 0.7 hours; 95% confidence intervals 0.0 to 1.4 hours in a 24-hour period).Conclusions: The results of this study show that recovery postappendectomy can be quantified by the measurement of uptime and that children undergoing a laparoscopic procedure may recover marginally more quickly than those undergoing an open procedure.     

Midgut atresias result from abnormal development of the notochord in an Adriamycin rat model

Background/Purpose: Prenatal exposure to Adriamycin in a rat model (ARM) has been reported to lead to a spectrum of tracheoesophageal and associated malformations of the gastrointestinal tract, including multiple intestinal atresias. An abnormal relationship of the notochord with the foregut has been implicated in the formation of esophageal atresias. The authors hypothesised that midgut atresias arise from abnormal notochord development in the region of the midgut. This study was designed to examine the gut-notochord relationship during early embryonic development. Methods: Timed pregnant Wistar rats were given 1.75 mg/kg of Adriamycin intraperitoneally on days 7, 8, and 9 of gestation. Embryos were recovered at 12-hour intervals from days 9.5 to 14, and at term. A control group was given saline instead of Adriamycin. Embryos were embedded in resin or wax, sectioned, and studied using light microscopy, paying particular attention to the notochord and surrounding structures. Results: The notochord appeared identical in controls and experimental embryos on day 9.5. However, on day 10.5 the notochord was diffusely abnormal in ARM, distorted, and tethered to foregut as well as midgut compared with controls. This abnormality was not seen in control embryos. On day 12 the notochord abnormalities were more exaggerated in the region of the midgut in ARM embryos. Full-term ARM animals had esophageal and multiple intestinal atresias. Conclusions: The notochord is abnormal in the region of the developing midgut, and this may account for the occurrence of atresias found in this region.     

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.     

Gastroesophageal reflux after repair of congenital diaphragmatic hernia

Background/Purpose: Mechanisms of postoperative gastroesophageal reflux (GER) remain unclarified in congenital diaphragmatic hernia (CDH). The aim of this study was to examine clinical features of CDH contributing to GER. Methods: GER was evaluated as esophageal acid exposure measured with 24-hour pH monitoring at the average age of 1.7 [plusmn] 0.9 months in 26 survivors of CDH. Correlations between the percentage total time pH below 4.0 (reflux index: RI)/number of long acid reflux (long reflux) and clinical features were investigated. The subjects were divided into 2 groups; group A: RI [le ] 4.0% (n = 7) and group B: R greater than 4.0% (n = 19). Clinical features were compared between the 2 groups. Results: No significant correlation was noted between RI/long reflux and birth weight, preoperative AaDO2, duration of artificial ventilation, and dimension of the defect. The incidence of direct closure was significantly higher, but use of nitric oxide inhalation and a presence of intrathoracic liver lobe herniation were significantly lower in group B. There was no significant difference of other clinical features indicating the severity of CDH between the 2 groups. Conclusion: An association of GER is not rare in early infancy after repair of CDH, which is influenced by the repair methods.     

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.