Esophageal Surgery in Newborns, Infants and Children

Transcription

1 Symposium on Pediatric Surgery I Esophageal Surgery in Newborns, Infants and Children Prema Menon and K.L.N. Rao Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India ABSTRACT The most common surgery on the esophagus by pediatric surgeons the world over is performed in the newborn period in babies with congenital esophageal atresia with tracheo-esophageal fistula. Post-operative complications like recurrent fistula, anastomotic stricture and some patients with gastroesophageal reflux would also require surgical intervention. Apart from esophageal dilatation, gastrostomy and feeding jejunostomy, children with strictures secondary to caustic ingestion, reflux or previous esophageal anastomosis may require esophageal substitution. This operation may also be required in babies with pure esophageal atresia as well as those with a long gap esophageal atresia with fistula. The entire stomach, stomach tubes, colon or jejunum are often used but techniques preserving as much of the original esophagus as possible are preferable and more physiological. Surgery is also required in children with congenital esophageal stenosis and duplication cyst. [Indian J Pediatr 2008; 75 (5) : ] klnrao@hotmail.com Key words : Esophageal surgery; Esophageal atresia; Esophageal replacement; Esophageal stricture; Thoracoscopy; Esophagoplasty; Neonate; Child Esophageal surgery is performed in all age groups in children. The most common indication, esophageal atresia (EA) is seen all over the world, especially so in our country. In fact, surgery for EA is the most common emergency surgery in neonates at our center, with about 180 cases per year. Healthy infants without pulmonary complications or other major anomalies can undergo primary repair in the first few days of life. Prompt diagnosis with appropriate clinical management and expeditious referral to a tertiary care center have led to survival rates in this group of 100% percent. 1 Due to the sheer numbers, and inadequate intensive care facilities in many parts of the country, overall results are not comparable with western centers. Delayed presentations are common but a lot of progress has been made over the past few decades in units dedicated to neonatal surgery even in this group. 2 Long term follow-ups are available even in babies who presented as late as on day 17 of life. 3 While congenital anomalies form the bulk of the indications for pediatric esophageal surgery, replacement of the esophagus for acquired lesions like caustic strictures are also performed, the techniques used being very similar to those in adults. Surgery in Neonate with Esophageal Atresia Correct preoperative management of a baby with EA Correspondence and Reprint requests : Dr. K.L.N.Rao, Professor and Head, Department of Pediatric Surgery, Advanced Pediatric Center, P.G.I.M.E.R., Chandigarh, , India. Ph: Ext (Off), (Res), (M); Fax: , forms an important part of the overall care. 4 The baby should be nursed with the chest in the upright position and the oropharynx and upper pouch repeatedly suctioned. Intravenous fluids, oxygen by hood and broadspectrum antibiotics are started. If there are features of respiratory failure, the baby is intubated. Bag-mask ventilation is not appropriate since it may cause acute gastric distention requiring emergency gastrostomy. Chest radiographs should be evaluated carefully for skeletal abnormalities, cardiovascular malformations, pneumonia, diaphragmatic hernia and a right aortic arch. Before surgery, it is important to examine the baby s abdomen and perineum. An abdominal radiograph to detect distal gas should be done as the surgical approach would not involve a thoracotomy in babies with pure EA. This is also evaluated for skeletal abnormalities, intestinal obstruction and malrotation. A contrast upper gastrointestinal series is not recommended. An echocardiogram and renal ultrasonogram should be obtained. The baby should be shifted to an intensive care unit and operated after proper evaluation and stabilization is achieved. The survival rate in babies with low birth weight, pneumonia or other major anomalies is lower with cardiac anomalies being the main cause of death. The Waterston classification appears to be still relevant as a prognostic indicator in our setup. 5 These babies are managed with parenteral nutrition, gastrostomy to prevent reflux of gastric contents through the fistula into the trachea and upper pouch suction until they are appropriate surgical candidates. Indian Journal of Pediatrics, Volume 75 September,

2 Prema Menon and K.L.N. Rao Bronchoscopy prior to starting of the procedure is performed in many centers. A prospective study in our department showed that it helped in identifying the upper pouch fistula, distance of the lower pouch fistula from the carina thereby helping placement of endotracheal tube beyond the fistula during surgery as well as identifying tracheomalacia. Although postoperative pulmonary complications and ventilator requirements were reduced, it did not alter the mortality rate. 6 Surgical repair is performed under general anesthesia with endotracheal intubation through a right posterolateral thoracotomy. Frequent desaturation can occur during surgery especially while retracting the lung. An experienced anesthetist is therefore essential as these babies should be manually ventilated with low inspiration pressures and small tidal volumes. The azygos vein is usually the first structure to be identified after thoracotomy. As it lies over the tracheoesophageal fistula, it is routinely ligated and divided. In a recent study, it has been suggested that by preserving the azygos vein, early postoperative edema of the esophageal anastomosis can be prevented resulting in a significant reduction in the number of anastomotic leaks. 7 Post-operatively babies are nursed in a TEF chair, so that elevation of the head end is always maintained at an angle of o. 8 We routinely start feeds on the 2 nd postoperative day through a trans-anastomotic nasogastric tube which has been inserted at the time of surgery. 4 In long gap EA, babies often end up with an initial gastrostomy and esophagostomy. Many complications are encountered with a gastrostomy before the patient gets an esophageal replacement. Although, it is routinely advised to minimally dissect the lower pouch because of its perceived deficiency of blood supply and to preserve the vagal fibres, mobilization of the lower pouch can bridge the defect in the first attempt and is far more physiological than an esophageal replacement. In a prospective, randomized study in our department, we compared 20 neonates with long gap EA and TEF managed by ligation of fistula, distal pouch mobilization and primary repair versus ligation of fistula, esophagostomy and gastrostomy followed by delayed esophageal replacement. 9 The mean duration of hospitalization as well as final survival was statistically significant in favor of the first group (p<0.05). Complications after esophageal atresia repair and their management Most neonates who undergo repair of EA and TEF have some degree of esophageal dysmotility which resolves with time. Strictures at the site of the anastomosis are common and may subsequently require dilatation. Serial esophagography should be performed at two months, six months and one year of age, or whenever swallowing difficulties occur. Recurrent tracheoesophageal fistula would require surgical correction and is mostly seen at the site of the primary anastomosis. Using a ventilating bronchoscope, the fistula is cannulated with a ureteric catheter which is then identified through esophagoscopy. 10 Tissue damage of the poorly vascularized distal esophagus and surgical dissection performed too close to the trachea have been postulated as risk factors. Approximately one half of patients with surgically corrected EA develop gastroesophageal reflux disease (GERD). Of these, one half responds to routine medical therapy with prokinetic agents, histamine H2 receptor blockers, or both, while the other requires surgical intervention in the form of fundoplication for correction. Long-term endoscopic follow-up may be indicated in these patients to look out for Barretts esophagus and its sequelae. In a prospective study in our unit over a 2 year period, 27 babies developed anastomotic leak. Creation of a feeding jejunostomy in 71% of the patients allowed us to maintain nutrition and also reduced the percentage of feeds in intercostal chest drain on an average from 25% to 8 %, thus reducing chest contamination. There is a high incidence of GER in these babies and an additional gastrostomy gave better results than feeding jejunostomy alone. (Monika Bawa, MCh thesis. Efficacy of management protocol for anastomotic leak after repair of EA and TEF, PGIMER. 2007). Esophageal stricture A contrast esophagogram is essential to know the anatomy of the esophagus, presence of multiple strictures as well as capacity and drainage of the stomach. In patients with associated GER, this should be first managed surgically before any definitive procedure on the esophagus. Most respond well to further esophageal dilatation. Localized strictures would require resection and anastomosis. During dilatation, if an esophageal opening cannot be found through endoscopy, a minilaparotomy and gastrostomy followed by retrograde dilatation should be attempted before opting for organ replacement / bypass procedures. Long strictures especially secondary to caustic ingestion often require esophageal replacement. Esophageal Replacement Esophageal replacement is indicated in children with pure EA, long-gap EA when anastomosis is not possible, corrosive strictures and other unusual causes. Type and location of the graft depend on etiology and surgeon preferences. Several options are now available with good results, such as use of the native esophagus or replacement with colon, stomach, or small bowel. Common problems with all esophageal substitutions include high stricture rate, leaks, GER and nutritional problems. An optimal substitute for the esophagus should 940 Indian Journal of Pediatrics, Volume 75 September, 2008

3 Esophageal Surgery in Newborns, Infants and Children preserve the native esophagus, have normal caliber, no space occupying lesion in the mediastinum, preserve gastric reservoir function, should withstand acidity and have a shorter suture line. Spitz et al recently published their results of 173 gastric transpositions through the posterior mediastinal route. 13 They observed that stomach functions as a conduit rather than as a reservoir. Nutritional problems as well as restricted pulmonary function appear to be common features. Native esophageal reconstruction is the procedure of choice rather than esophageal replacement. A long-term retrospective review of 21 out of 26 infants with pure EA who underwent delayed primary anastomosis between 1977 and 2004 showed that this provides excellent functional results in spite of the high incidence of GER (66%) with 43% of these needing fundoplication. 12 Sixteen children developed strictures at the anastomotic site; 10 responding to repeated dilatations while 6 needed resection and reanastomosis. At the end of the study period, 15 out of the 17 survivors (88%) were on normal diet with no respiratory problems while 2 (12%) were dependent on gastrostomy feeds. There were four deaths (19%) in this group. This method of management is based on the natural growth of the esophagus with the interim period managed by upper pouch suctioning and nutritional support. The median age at operation was 80 days and the median hospital stay was 5.5 months which may not be feasible in our setup. Reconstructive methods using gastric tubes are popular. Preparation of patient for gastric tube esophagoplasty includes pre-operative contrast study via gastrostomy or barium swallow, dietary advice i.e. high protein diet with evidence of weight gain, as well as assessment and management of co-existent cardiac and pulmonary lesions. Sham feeding should always be started after esophagostomy to develop oral feeding mechanisms early and shorten time to complete oral feeding after delayed esophageal repair. Counseling of parents regarding 2 procedures, i.e. gastric tube esophagoplasty followed by closure of esophageal ends approx 6 weeks later, duration of hospital stay which is likely to be more after the 2nd surgery, likely anesthetic complications, chest complications, as well as possibility of esophageal dilatations after completion of all stages should be done well in advance. We first reported our technique of fundal tube esophagoplasty in 2003 in 4 patients where the entire native lower esophagus is retained with the remaining required length created out of the fundus as a tube in continuity. 13 This is not possible with any other substitute except interposition grafts. The tube is brought out through the neck in a retrosternal fashion. Postoperatively a chest X-ray should be performed immediately after surgery (day 0). A few weeks later, a contrast study is done through the neck stoma followed by esophago-esophageal anastomosis. Since native esophagus is used, there is less chance of peptic ulceration at the anastomotic site. More of the stomach is available increasing intake and reducing chances of reflux. After closure of the neck stomas, oral contrast study to see caliber, stenosis, leak, stricture, reflux and stomach capacity is performed. Clinically, patients should be assessed for improvement in growth percentiles, developmental milestones, respiratory tract assessment, ability to take solids and liquids orally without difficulty ad libitum. We have so far ( ) performed this procedure successfully in 28 patients having pure EA (9), EA/ TEF with leak or long gap (16) and caustic strictures. 3 Seven patients had prolonged salivary leak which was managed conservatively with nutritional management and esophageal dilatation with only one requiring excision of fistula tract. Long-term complications have included stricture in 3 cases requiring local resection in 2 and conversion to colon interposition in 1. Dysphagia (5) mostly improved with time and 1-2 esophageal dilatations. Reflux related problems were managed with head up position, H2 blockers for 2 years at least and fundoplication in all patients. Mobilization of esophagus is sometimes difficult in patients with caustic strictures and post leaking EA/TEF. Additional thoracotomy was required in 2 children with caustic esophageal strictures. Poorly placed gastrostomy tube with injury to gastroepiploic arcade, odd location of cervical esophagostomy, intraabdominal adhesions and small capacity stomach create additional problems during creation of the fundal tube. Gastric tubes are simple to construct and long lengths can be created. Neo angle of His can be created, and the distal part of the tube remains in the high pressure abdominal zone reducing chances of reflux. Fundoplication can also be easily added to the procedure. But anastomosis between cervical esophagus and antral gastric mucosa increases likelihood of peptic ulceration at anastomotic site. These reversed gastric tubes require good stomach capacity so that sufficient length of tube to reach the neck can be created, normal stomach drainage and motility and availability of left gastroepiploic artery and arcade. Advantages over colonic interposition include rich blood supply, no tension on the interposed segment, resistance of gastric mucosal tube to acid ulceration and no bowel complications like diarrhea. Complications of reversed gastric tube include cervical anastomotic leak, stricture, narrowing at diaphragmatic hiatus, smaller feeds due to reduced stomach capacity, acid reflux and psychological difficulties. Our own long term results of reversed gastric tube (n=10), show that although they require multiple esophageal dilatations, and have prolonged neck leak, ultimately almost all patients do well and are able to take solids orally without difficulty. In a series of 21 children who had isoperistaltic gastric tube for varied indications, 16 could accept a normal diet, Indian Journal of Pediatrics, Volume 75 September,

4 Prema Menon and K.L.N. Rao 2 had significant dysphagia and 3 needed a feeding jejunostomy. The esophagogastric anastomosis leaked in 2 cases, but both closed spontaneously. A temporary dumping syndrome was encountered in two children. Two patients had strictures of their upper anastomosis responding to dilatations. The two patients who had a pharyngogastric anastomosis developed either intractable stricture or nonfunctioning anastomosis. One of them died 9 months later from aspiration pneumonitis. Two patients had cervical Barrett s esophagus above the anastomosis, indicating the need for lifelong endoscopic follow-up. 14 Hadidi has developed a simple technique to improve the results of colon replacement of the esophagus in children. 15 In 5 neonates with long gap EA with or without fistula and another 6 with long segment caustic esophageal stricture, at the gastrostomy operation, the segment of colon to be used for replacement was chosen and the trunk of the corresponding vessel say middle colic artery supplying the transverse colon was ligated and divided proximal to the marginal artery. One to three months later, this colon was used for esophageal replacement. The follow-up ranged from months. By increasing the blood supply to the transverse colon through the left upper colic and marginal vessels, the success rate of colonic replacement improved and minimized morbidity. Only 1 patient developed stricture at the colo-esophageal anastomosis which required resection and anastomosis six months later. Thoracoscopy in EA and other esophageal surgery The thoracoscopic approach to the treatment of EA is being increasingly performed. Secondary effects like thoracic cage deformities, winged scapula, or scoliosis are reduced in comparison to the open technique. In a study of 51 neonates with EA managed thoracoscopically, the operative procedure took minutes (mean 178 minutes) which is understandable. However, the stenosis rate (45%) appears to be much higher than with the conventional open technique. Postoperative leakage occurred in 9 patients (18%). In this study, 6 patients also underwent thoracoscopic aortopexy for tracheomalacia. In 2 patients the thoracoscopic procedure had to be converted to a thoracotomy. 16 In a multi-institutional analysis of 104 newborns with a mean weight of 2.6 kg (±0.5), who underwent thoracoscopic repair of EA, the mean operative time was minutes ±55.5 with 11.5% infants developing an early leak or stricture at the anastomosis. 17 Thirty three (31.7%) required esophageal dilatation at least once. Conversion to an open thoracotomy was done in 5 cases. However, 25 newborns (24.0%) later required a laparoscopic fundoplication, the incidence of which appears to be much higher than with the open technique. Three patients died, one related to the EA/TEF on the 20th postoperative day. Although cosmetic results may be better, no other major advantage has been demonstrated by the laparoscopic route so far. Compared to adults, achalasia is rare in children. Laparoscopic Heller myotomy with a partial fundoplication is presently the gold standard for managing this condition in children. 18 Other causes of esophageal obstruction While evaluating a newborn with drooling of saliva and respiratory distress, diagnosis other than EA should be kept in mind. Traumatic instrumentation can produce pharyngeal or upper esophageal perforation and an attempt at passage of a catheter may produce a false passage submucosally or a pseudodiverticulum. 19 In the absence of traumatic instrumentation a spontaneous perforation of esophagus should be considered. Mostly the mucosal tear heals spontaneously, however, the risk of mediastinitis does exist. Rarely, thoracotomy may be indicated. Esophagoscopy is contraindicated in these cases as it may actually increase the size of the perforation. Babies should be monitored for leukocytosis, fever and swallowing abnormalities. Obstruction of the lower end of esophagus can occur rarely due to congenital esophageal stenosis which may respond to bouginage, endoscopic excision of diaphragm or surgical resection anastomosis. Enteric duplication cysts can occur in relation to the wall of the esophagus rarely communicating with its lumen. They can usually be removed preserving the esophageal integrity by thoracotomy or thoracoscopy. Esophageal intramural pseudo-diverticulosis is another extremely rare entity in children associated with hiatus hernia and benign esophageal stricture secondary to GER. Patients present with intermittent but slowly progressive dysphagia especially for solids. They symptomatically respond to esophageal dilatation and fundoplication. 20 CONCLUSIONS Esophageal surgery in neonates requires high degree of surgical skills and good neonatal postoperative nursing care. Post-operative problems are very common after any form of esophageal replacement but success can be achieved in almost all cases. However, the surgeon has to be passionate towards the baby, tenacious and also give moral support to parents who may get disillusioned with the time taken for ultimate recovery. This is the most important aspect for the surgeons who wish to care for these children with esophageal substitutions. REFERENCES 1. Bendi G, Chowdhary SK, Rao KLN. Esophageal atresia with tracheoesophageal fistula: an audit. J Indian Assoc Pediatr Surg 2004; 9: Indian Journal of Pediatrics, Volume 75 September, 2008

5 Esophageal Surgery in Newborns, Infants and Children 2. Narasimharao KL, Joglekar A, Mitra SK, Pathak IC. Oesophageal atresia: delayed presentation and survival. J Indian Med Assoc 1985; 83 : Menon P, Samujh R, Rao KLN. Esophageal atresia. Indian J Pediatr 2005; 72 : Menon P, Rao KLN. Management of esophageal atresia and tracheoesophageal fistula. Bulletin of Pediatr Gastroenterol Hepatol Nutr 2003; 1: Eradi B, Narasimhan KL, Rao KLN et al. Waterston classification revisited-its relevance in developing countries. J Indian Assoc Pediatr Surg 2003; 8 : Pimpalwar A, Kaplish B, Rao KLN, Puri GD, Mitra SK. Esophageal atresia: Is routine bronchoscopy before thoracotomy useful? J Indian Assoc Pediatr Surg 1997; 2 : Sharma S, Sinha SK, Rawat JD, Wakhlu A, Kureel SN, Tandon R. Azygos vein preservation in primary repair of esophageal atresia with tracheoesophageal fistula. Pediatr Surg Int 2007; 23: Kalia R, Menon P, Rao KLN. Nursing care for the surgical neonate. J Neonatology 2005; 19: Tripathy PK, Rao KLN, Chowdhary SK et al. Lower pouch mobilization in long gap esophageal atresia. J Indian Assoc Pediatr Surg 2004; 9 : Narasimhan KL, Rao KLN, Mitra SK. Management of recurrent tracheoesophageal fistula. Indian Pediatr 1990; 27 : Sri Paran T, Decaluwe D, Corbally M, Puri P. Long-term results of delayed primary anastomosis for pure oesophageal atresia: a 27-year follow up. Pediatr Surg Int 2007; 23 : Rao KLN, Menon P, Samujh R, Chowdhary SK, Mahajan JK. Fundal tube esophagoplasty for esophageal reconstruction in atresia. J Pediatr Surg 2003; 38 : Spitz L, Kiely E, Pierro A. Gastric transposition in children: a 21 year experience. J Pediatr Surg 2004; 39 : Borgnon J, Tounian P, Auber F et al. Esophageal replacement in children by an isoperistaltic gastric tube: a 12-year experience. Pediatr Surg Int 2004; 20 : Hadidi A. A technique to improve vascularity in colon replacement of the esophagus. Eur J Pediatr Surg 2006; 16 : van der Zee DC, Bax KN. Thoracoscopic treatment of esophageal atresia with distal fistula and of tracheomalacia. Semin Pediatr Surg 2007; 16 : Holcomb GW III, Rothenberg SS, Bax KMA et al. Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula, a multi-institutional analysis. Ann Surg 2005; 242 : Mattioli G, Esposito C, Prato AP et al. Results of the laparoscopic Heller-Dor procedure for pediatric esophageal achalasia. Surg Endosc 2003; 17: Narasimharao KL, Sachdeva S, Narang A et al. Perforation of pharynx mimicking esophageal atresia. Indian Pediatr 1985; 22 : Garg K, Katariya S, Narasimharao KL, Pathak IC. Esophageal intramural pseudo-diverticulosis. Indian Pediatr 1985; 22 : Indian Journal of Pediatrics, Volume 75 September,