The survival of infants born with esophageal atresia. Esophageal Atresia and Tracheoesophageal Fistula: Surgical Experience Over Two Decades

Size: px
Start display at page:

Download "The survival of infants born with esophageal atresia. Esophageal Atresia and Tracheoesophageal Fistula: Surgical Experience Over Two Decades"

Transcription

1 Esophageal Atresia and Tracheoesophageal Fistula: Surgical Experience Over Two Decades Josephine Y. Tsai, MD, Leah Berkery, BS, David E. Wesson, MD, S. Frank Redo, MD, and Nitsana A. Spigland, MD Department of Surgery, The New York Hospital-Cornell Medical Center, New York, New York Background. Despite improvements in survival, for infants born with esophageal atresia tracheoesophageal fistula, or both, the morbidity associated with repair of these anomalies remains high. Methods. This report retrospectively analyzes 81 patients with esophageal atresia, tracheoesophageal fistula, or both presenting to our institution between 1975 and 1995, with a focus on anastomotic complications. Results. There were 46 male and 35 female patients with a mean gestational age of 37 weeks and mean birth weight of 2443 g. Forty-four patients underwent primary esophageal anastomoses, 7 underwent delayed primary anastomoses, 12 patients underwent staged repairs, and 5 underwent repair of H-type fistulas. Among 62 patients with anastomoses, complications included stricture in 25/62 patients (40%), leakage in 12/62 patients (19%), and recurrent tracheoesophageal fistulas in 6/62 patients (10%). Stricture rates for esophagocolonic anastomoses versus esophagoesophageal anastomoses were 4/8 cases (50%) versus 21/54 cases (39%). This difference was not statistically significant. All esophagoesophageal strictures were managed successfully with dilations; three of four esophagocolonic strictures required anastomotic revision. The leakage rate for esophagocolonic anastomoses versus esophagoesophageal anastomoses was 6/8 cases (75%) versus 6/54 cases (11%). This difference was statistically significant (p ). Two patients required revision of their colon grafts secondary to necrosis. Eighteen of 81 patients (22%) died. Operative mortality was 9/74 (12%). Causes of death included associated anomalies (n 15), recurrent aspiration and sepsis secondary to missed fistula (n 1), and unknown (n 2). Conclusions. Although the morbidity associated with surgical repair of these anomalies is high, this does not affect the overall survival. The high complication rate associated with colonic interposition suggests that one should preserve the native esophagus as a primary conduit whenever feasible. (Ann Thorac Surg 1997;64:778 84) 1997 by The Society of Thoracic Surgeons The survival of infants born with esophageal atresia (EA), tracheoesophageal fistula (TEF), or both has improved dramatically since Cameron Haight s first successful repair in 1941 [1]. Improvements in survival are largely attributable to refinements in neonatal intensive care, anesthetic management, ventilatory support, and surgical techniques. Survival may now be achieved in infants with low birth weight [2], with mortality limited to those patients who have severe life-threatening anomalies. Despite improvements in survival, the morbidity associated with surgical repair of these anomalies remains high [3]. In this review, we update our previously reported experience with EA and TEF [4]. We retrospectively analyzed the charts of 81 patients with EA, TEF, or both who presented to our institution over the past two decades, with an emphasis on anastomotic complications so that future determinations could be made to minimize morbidity and mortality. Presented at the Poster Session of the Thirty-third Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Feb 3 5, Address reprint requests to Dr Spigland, Division of Pediatric Surgery, The New York Hospital-Cornell Medical Center, 525 E 68th St, F763, New York, NY ( naspigla@mail.med.cornell.edu). Material and Methods Between 1975 and 1995, 81 patients with EA, TEF, or both were treated at the New York Hospital-Cornell Medical Center. The data were collected retrospectively from hospital charts. The information obtained was compared with data from a previous review from our institution published in 1975 [4]. Fisher s exact test was used for comparative statistical analysis with a significance level of p less than In our current study there were 46 male patients (57%) and 35 female patients (43%). The mean gestational age was 37 weeks (range, 28 to 42 weeks). The mean birth weight was 2,443 g (range, 915 to 4,035 g). The mean Apgar score at 1 minute was 7, and the mean at 5 minutes was 8. All cases were classified into anatomic anomaly type based on the Gross classification [5] (type A-D, plus H-type TEF). The most common variant was EA with a distal TEF (type C), which occurred in 67 patients (83%). Pure EA (type A) was present in 7 patients (9%). Five patients had an H-type TEF without EA (6%). One infant (1%) had EA with proximal TEF (type B), and 1 infant (1%) had both a proximal and distal TEF (type D). Infants were also assigned to risk groups A, B, or C as described by Waterston and associates [6]: 1997 by The Society of Thoracic Surgeons /97/$17.00 Published by Elsevier Science Inc PII S (97)

2 Ann Thorac Surg TSAI ET AL 1997;64: ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA 779 Group A 1. Birth weight 2,500 g and well Group B 1. Birth weight 1,800 to 2,500 g and well, or 2. Birth weight 2,500 g but moderate pneumonia and other congenital anomaly Group C 1. Birth weight 1,800 g or 2. Birth weight 1,800 g with severe anomaly or pneumonia Twenty-four patients (30%) were classified in group A, 31 patients (38%) in group B, and 26 patients (32%) in group C. Forty of 81 patients (49%) had associated congenital anomalies. Congenital cardiac anomalies were the most frequent, occurring in 24 (30%) of 81 patients. These included atrial septal defects (ASDs), ventricular septal defects (VSDs), patent ductus arteriosus, patent foramen ovale, dextrocardia, pulmonic stenosis, subaortic stenosis, bicuspid aortic valve, hypoplastic left ventricle, and tricuspid atresia. Gastrointestinal anomalies occurred in 14 of 81 patients (17%), and included imperforate anus, duodenal atresia, pyloric atresia, biliary atresia, and annular pancreas. Major renal anomalies occurred in 9 of 81 patients (11%) and included hydronephrosis, renal agenesis, double collecting system, and horseshoe kidney. Minor skeletal deformities occurred in 8 of 81 patients (10%) and included missing radius, polydactyly, and 13th rib. Chromosomal anomalies occurred in 7 (9%) of 81 patients: trisomy 18 in 4 patients (5%), trisomy 21 in 2 patients (2%), and fragile X syndrome in 1 patient (1%). Severe life-threatening anomalies occurred in 8 of 81 patients (10%): bilateral renal agenesis and hypoplastic lung in 1 patient (1%), trisomy 18 in 4 patients (5%), dextrocardia, ASD, pulmonic stenosis, and left renal agenesis in 1 patient (1%); dextrocardia, ASD, VSD, imperforate anus, hypoplastic left lung, and agenesis of right lung in 1 patient (1%); and hypoplastic left heart and ASD in 1 patient (1%). Among 81 patients in our series, 7 died before surgical intervention, of severe associated congenital anomalies. Four patients died of severe anomalies during staging procedures, and 2 patients were transferred to other institutions after preliminary gastrostomy or esophagostomy. Of the remaining 68 patients, 44 patients underwent primary esophageal anastomoses, 7 patients underwent delayed primary anastomoses, and 5 patients underwent repair of H-type fistulas. Twelve patients underwent staged repairs for long-gap type A and C anomalies including esophageal anastomosis after bougienage (2 patients), colon interposition (8 patients), and staged esophageal lengthening (2 patients; 1 with anastomosis). Primary repair included fistula division and end-toend esophagoesophageal anastomoses within the first 48 hours. Delayed primary repairs included fistula division and esophageal anastomoses after 48 hours (median time, 9 days) after an initial gastrostomy. These were performed in patients with severe pneumonia or other anomalies that prohibited an immediate definitive operation. Staged repairs included initial thoracotomy and fistula division with gastrostomy or esophagostomy, and delayed esophageal anastomosis or colon interposition. In most cases, the position of the aortic arch was confirmed by cardiac echocardiography preoperatively and thoracotomy was performed opposite the side of the aortic arch. Primary repairs were performed by a retropleural approach whenever feasible. A single layer endto-end esophageal anastomosis was constructed using interrupted 5-0 silk sutures. Staged repairs were performed in 12 cases of wide-gap type C or type A anomalies (gap length greater than two vertebral bodies or greater than 3 cm). The mean gap length for the wide-gap anomalies in our series was four vertebral bodies. After initial thoracotomy with fistula division or gastrostomy, bougienage lengthening of the upper esophageal pouch was begun daily. Despite continuous sump drainage of the upper pouch with bougienage lengthening, 10 patients underwent cervical esophagostomies because of repeated bouts of aspiration. These patients subsequently underwent colon interposition or primary esophageal anastomoses after multistaged extrathoracic esophageal lengthening. Colon interpositions usually involved the right colon including the ileocecal valve in an isoperistaltic, substernal fashion. Follow-up of patients in our series ranged from 2 weeks to 9 years. Results Anastomotic strictures occurred in 25 of 62 patients (40%) with esophagoesophageal (EE) or esophagocolonic (EC) anastomoses. A stricture was defined as an anastomotic narrowing on an upper gastrointestinal (UGI) series that required one or more dilations. The median number of postoperative dilations performed in our series was two with a range of one to nine dilations. Nine of 25 patients (15%) required more than two dilations. Nine of 25 patients (15%) with strictures had associated anastomotic leaks. Anastomotic leaks were present in 12 of 62 patients (19%), and subsequent strictures that required dilation developed in 9 of 12 patients (75%) with anastomotic leaks. Five of 25 patients with strictures (20%) had associated gastroesophageal reflux (GER). The stricture rate in the staged group was 5/11 patients (45%) versus 20/51 patients (39%) for the primary and delayed primary groups. These differences were not statistically significant. The stricture rates for EC anastomoses versus EE anastomoses were 4/8 cases (50%) versus 21/54 cases (39%). These differences were also not statistically significant. All EE strictures were managed successfully with dilations; 3/4 EC strictures required anastomotic revision. Anastomotic leaks were identified clinically by the presence of saliva in the chest tube, and confirmed by UGI series. The leak rate in the staged repair group (wide-gap type C and type A) was 6/11 patients (54%) versus 6/51 patients (11%) in the primary and delayed primary group. These differences were statistically significant (p 0.004). The leakage rates for EC anastomoses versus EE anastomoses were 6/8 cases (75%) versus 6/54 cases (11%). These differences were also statistically sig-

3 780 TSAI ET AL Ann Thorac Surg ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA 1997;64: Fig 1. Comparison of leak and stricture rates in the current series ( ) as compared with the previously reported series [4] ( ). These differences were not statistically significant. nificant (p ). Among the 12 patients with anastomotic leaks, 3 of the 6 with EE leaks (50%) developed strictures, which were successfully managed by dilation. Five of the six EC leaks (83%) eventually required surgical revision for intractable stricture or graft necrosis. A comparison of the leak and stricture rates between the previous review from our institution and the current series is seen in Figure 1. The differences were not statistically significant. Among 8 patients who underwent colon interposition, strictures developed in 4 of 8 patients (50%) anastomotic leaks developed in 6 of 8 patients (75%), and 5 of 8 patients (62%) required reoperation. Indications for reoperation included nondilatable strictures in 3 of 8 patients (37%) and colon graft necrosis in 2 of 8 patients (25%). The 2 patients in whom colon graft necrosis developed underwent secondary surgical procedures using the left colon and transverse colon, respectively. Recurrent tracheoesophageal fistulas were diagnosed in 6 patients at 10 days, 1 month, 1 month, 3 months, 1 year, and 4 years postoperatively by UGI series and confirmed by bronchoscopy. Three of these patients were treated by reoperation, which involved fistula division with interposition of a pleural flap between the suture lines. In 2 patients the follow-up management is unknown. One patient was managed nonoperatively for a pinpoint fistula noted on bronchoscopy. Two patients had missed fistulas (2.5%). One of these patients was presumed to have a type A anomaly, but later was discovered to have a type B anomaly. His postoperative course was complicated by pneumonia, and a missed fistula was discovered on UGI series. He underwent successful reoperation and division of his proximal TEF on postoperative day 7. In the second patient, recurrent episodes of pneumonia developed. A UGI series performed 1 month postoperatively did not reveal the presence of fistulas. The patient subsequently died 6 months postoperatively of recurrent bronchopulmonary pneumonia and sepsis. Two missed fistulas were noted on autopsy. Gastroesophageal reflux was documented by UGI series or ph probe in 9 patients. Gastroesophageal reflux was diagnosed between 1 month and 6 years postoperatively. Six of these patients were treated medically. Three of the 9 patients required antireflux procedures. Tracheomalacia was diagnosed in 9 patients (11%) by bronchoscopy between 1 month and 3 years postoperatively. Two patients manifested dying spells requiring a surgical procedure (aortic suspension in 1, stent placement in 1). The remaining 7 patients had less severe symptoms and were managed nonoperatively. Eighteen of 81 patients (22%) died. Seven patients died before any surgical intervention of known severe associated anomalies (trisomy 18 in 1; dextrocardia, ASD, VSD, hypoplastic lungs, and imperforate anus in 1; dextrocardia, ASD, pulmonic stenosis, and renal agenesis in 1; subaortic stenosis in 1; and bilateral renal agenesis and hypoplastic lungs in 1) and unknown causes (n 2). Four patients died after preliminary staging procedures (eg, gastrostomy, thoracotomy with fistula ligation) before definitive surgical repair. Causes of death included trisomy 18 in 2, VSD and bicuspid aortic valve in 1, and prematurity and intracranial hemorrhage in 1. Seven patients died after definitive surgical repair. The causes of death among these patients included recurrent aspiration and sepsis secondary to missed fistula in 1, trisomy 18 in 1, biliary atresia and liver failure in 1, hyaline membrane disease and bronchopulmonary dysplasia in 1, tricuspid atresia, VSD, and rudimentary right ventricle in 1; ASD, anamolous pulmonary venous return, and hypoplastic left heart in 1; and prematurity and subarachnoid hemorrhage in 1. The operative mortality was 9 of 74 patients (12%) who underwent surgical procedures. The late mortality was 2 of 74 patients (3%). One of these patients died at 1 year of age of liver failure secondary to biliary atresia. One patient with multiple cardiac anomalies died of an unknown cause at 2 years of age. If one considers only those infants who underwent definitive correction of their anomalies, overall mortality is 7 of 67 patients (10%) with an operative mortality of 5 of 67 patients (7%) and a late mortality of 2 of 67 patients (3%). A comparison of the total mortality, operative mortality, late mortality, and mortality after definitive repair between the two series is seen in Figure 2. The survival based on Waterston s criteria was 100% for group A, 89% for group B, and 11% for group C. Fig 2. Comparison of overall mortality, operative mortality, late mortality, and mortality after definitive (def.) repair in the current series ( ) as compared with the previously reported series [4] ( ). These differences were not statistically significant.

4 Ann Thorac Surg TSAI ET AL 1997;64: ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA 781 Comment An analysis of the surgical experience with EA and TEF at the New York Hospital-Cornell Medical Center over the past two decades, as compared with the report from our institution published in 1975 [4], shows that there were no statistically significant differences with respect to sex predominance, birth weight, proportion of infants with low birth weight, proportion of infants with major lifethreatening congenital anomalies, anomaly type, or type of operative repair. The respective anastomotic leak and stricture rates among both series were comparable. A higher leak rate (19% versus 10%) is noted in the current series, but this difference is not statistically significant. The overall mortality, operative mortality, and mortality rates after definitive repair are also comparable between both groups. The overall survival rates were 75% and 78%, respectively, and survivals for those infants undergoing definitive correction of their anomalies were 93% and 90%, respectively. Despite advances in neonatal intensive care as well as surgical and anesthetic techniques, a significant proportion of infants in both series died because of associated life-threatening congenital anomalies. Current trends toward early detection of complex genetic and chromosomal malformations in utero, early total correction of cardiac defects, and improved neonatal intensive care management of premature infants may help increase survival rates for patients in the Waterston C risk group. If we exclude those infants who died preoperatively or awaiting definitive repair after preliminary staging procedures, survival rates that exceed 90% may be achieved, which are comparable with those published among several large series [3, 7 9]. Despite improvements in survival over the past five decades ranging from 36% in the pre-1950 era [7] to as high as 95% [3] in 1995, the incidence of anastomotic complications remains consistently high, with leak rates varying from 8.5% to 36% [3, 7 15], stricture rates varying from 8% to 37% [3, 8 14, 16], and the rate of recurrent TEF varying from 3% to 12% [3, 7, 8, 11, 13], depending on the criteria used to define these complications. The use of silk sutures [10, 15], excessive anastomotic tension [8, 14], and excessive distal esophageal mobilization [17] have been implicated in the pathogenesis of anastomotic leaks. The anastomotic leak rate in our current series of 19% is comparable with that reported in other series [3, 8 15]. We also noted a statistically significant increase in leak rates among those infants with wide-gap anomalies who underwent staged repairs as compared with those infants who underwent primary esophageal repairs (54% versus 11%; p 0.004). Although gap length between the pouches and anastomotic tension are important factors associated with anastomotic morbidity, the more likely explanation for the increased leak rate in our series is that 8 of 11 patients (72%) who underwent staged definitive repairs had colon interpositions. We noted a significantly higher leak rate among patients who underwent EC anastomoses versus EE anastomoses (75% versus 11%; p ). Anastomotic leak rates associated with colon interposition range from 9% [18] in one series to as high as 70% [19]. Another large series encompassing 227 patients reports a complication rate of 68% with colon interposition [3]. The high incidence of leak associated with cervical EC anastomoses has been attributed to impaired blood supply in the cephalad end of the interposition graft [18]. Additionally, leaks associated with colon interposition had a high reoperation rate. Five of 6 esophagocolonic leaks (83%) required surgical revision for intractable strictures or graft necrosis. Our stricture rate of 40% is slightly higher than that reported in other series [3, 8 14, 16]. We attribute this to the fact that a stricture was defined in our series as an anastomotic narrowing seen on UGI series that required one or more dilations. Other series define strictures as those requiring greater than two [7], three [14], or four [3, 8, 10] dilations with stricture rates ranging from 15% to 35% in these series [3, 7, 8, 10, 14]. Nine patients (15%) in our series required more than two dilations. A number of predisposing factors have been implicated in the pathogenesis of anastomotic strictures including a two-layer anastomosis [7, 10], gap length [8, 14], anastomotic tension [8, 14], silk sutures [13, 16], anastomotic leak [13, 15, 16], and GER [3, 7, 9, 16, 19]. In 9 of 12 patients (75%) with anastomotic leaks in our series, subsequent strictures developed that required dilation. Chittmittrapap and associates, in their series of 199 patients [15], noted an increased stricture rate in those patients with anastomotic leaks (71%) versus those without leaks (36%). The stricture rates in our series for EC anastomoses versus EE anastomoses were 50% versus 39%, a difference that was not statistically significant. However, all EE strictures were managed successfully with dilations, whereas 3/4 EC strictures required anastomotic revision. We currently favor the use of Vicryl (Ethicon, Somerville, NJ) sutures as well as extensive mobilization of the upper esophageal pouch routinely, to minimize anastomotic tension. If excessive tension persists despite upper pouch mobilization we advocate performing circular myotomies as described by Livaditis [20], mobilizing the gastroesophageal junction, or performing a Collis gastroplasty [21] in selective cases. Spitz [22] has advocated prophylactically paralyzing and ventilating infants who had anastomoses performed with extreme tension for 5 days postoperatively to minimize disruptive anastomotic forces. Although there were no leaks in that series, the stricture rate was 72% and the rate of GER was 54%. Chittmittrapap and associates [16] showed that GER significantly increased the stricture rate. Another large series of 303 patients noted a 52% rate of GER in 87 patients with anastomotic stricture [9]. In our series 5 of 25 patients (20%) with strictures had associated GER. Our GER rate (11%) is lower than rates quoted in other series of 37.9% [7], 39% [19], and 58% [3]. We believe that the true incidence of GER is higher than that reflected in our series, and that the disparity arises from the difficulty associated with properly documenting and confirming the presence of GER during postoperative follow-up

5 782 TSAI ET AL Ann Thorac Surg ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA 1997;64: visits. We advocate aggressive diagnosis and treatment of GER to reduce postoperative stricture rates The reported incidence of recurrent TEF ranges between 3% [3] and 12% [13]. The incidence of recurrent TEF in our series was 10%. The pathogenesis of recurrent TEF has been an anastomotic suture line leak with erosion through the site of previous repair of the TEF [3]. Because recurrent TEFs rarely close spontaneously, we advocate reoperation with interposition of a pleural or pericardial flap after fistula division. Missed fistulas occurred in 2 patients (2.5%) in our series. One of these patients died of recurrent bronchopulmonary pneumonia and sepsis 6 months postoperatively. When the upper esophageal pouch cannot be distended with air on plain roentgenograms, nonionic water-soluble contrast medium should be used to opacify the proximal pouch, so that proximal fistulas may be recognized preoperatively. It is also extremely important to routinely dissect the proximal pouch to the thoracic inlet and to completely divide the common wall between the trachea and proximal esophagus to avoid overlooking proximal fistulas. One needs to have a high level of suspicion for this complication postoperatively because the symptoms associated with missed fistulas, such as aspiration and recurrent respiratory tract infections, may be attributed to GER or tracheomalacia. When recurrent or missed fistulas are strongly suspected clinically, cineesophagography may need to be repeated several times. Special techniques such as placing the patient in a prone position during contrast esophagography and injecting contrast medium at 2-cm increments while gradually withdrawing the nasogastric tube may help diagnose these entitities. If contrast studies have negative results we advocate simultaneous esophagoscopy and bronchoscopy with methylene blue injection or cannulation of the fistula. The association of tracheomalacia with EA and TEF has been well recognized, with a reported incidence ranging from 16% [13] to 33% [23]. The incidence of tracheomalacia in our series is 11%. The most serious problem associated with tracheomalacia is dying spells, which occurred in 2 patients in our series, who required aortopexy and stent placement respectively, for control of symptoms. When severe tracheomalacia is documented by bronchoscopy in symptomatic infants (apnea, cyanosis, recurrent pneumonia with expiratory obstruction of the trachea) surgical intervention should not be delayed. The management of newborns with isolated EA (type A) and long-gap type C anomalies is often complex and presents a challenge for pediatric surgeons. The operative morbidity associated with esophageal replacement procedures is high [3, 18, 19, 24]. In addition to anastomotic leaks, strictures, and graft ischemia, other late complications include GER, recurrent aspiration, esophageal dysmotility, swallowing problems, ulceration, bleeding, respiratory tract infections, tortuosity of the graft, and failure to thrive [3, 18, 24]. The increased morbidity has served as an impetus for developing new methods for bridging the gap between the esophageal segments for wide gap anomalies. Howard and Myers [25] first reported upper pouch bougienage with mercury-filled bougies as a method of elongating the upper pouch. Others have advocated esophageal autoanastomosis by producing a mucosalined fistula [26], lengthening of the upper pouch by circular myotomy [20], and electromagnetic esophageal bougienage [27]. Puri and colleagues [28] demonstrated that mechanical stretching of the pouches is not necessary because spontaneous growth and hypertrophy of the esophageal pouches occurs in the first months of life. Bensoussan and associates [29] reported 6 infants with wide-gap anomalies who underwent primary repair at 3 months of age after spontaneous elongation of the esophagus. Kimura and Soper [30] reported multistaged extrathoracic esophageal elongation in patients with cervical esophagostomies in an attempt to elongate the native esophagus and perform a primary repair. A review of the data obtained from several large series [8, 14, 24, 28, 31, 32] of patients with long-gap anomalies who underwent delayed EE anastomoses revealed leak rates that varied widely from 0% [32] to 100% [14] and stricture rates that ranged from 50% [8, 32] to 90% [24]. The high morbidity in these cases has been attributed to anastomotic tension. Despite the high complication rate reported for delayed EE anastomoses for long-gap anomalies, most authors [8, 14, 24, 28, 31, 32] favor primary EE anastomoses as compared with esophageal replacement. The long-term functional results were much better with EE anastomoses as compared with esophageal replacement procedures [24, 31, 32] (ie, dysphagia and feeding difficulty were associated with esophageal substitution). Additionally, despite the high incidence of anastomotic strictures associated with EE anastomoses in these patients [8, 14, 24, 28, 31, 32], most of these strictures responded to dilation and very few required surgical revision, as was the case in our series. In Rescorla and associates series [24], 2/4 EC strictures (50%) required surgical revision as compared with 2/9 EE strictures (22%). The overall incidence of secondary surgical procedures for colon grafts was also higher [24] when compared with the reoperation rate for EE anastomoses. The reoperation rate among colon grafts was also higher in our current series. In addition to leaks and strictures, operative indications included revision for colon redundancy [24], bleeding from ulceration [24], and colon fibrosis [14]. Life-threatening complications (eg, colon graft necrosis) occurred with a higher frequency among those patients who underwent EC versus EE anastomoses [24], as was the case in the present series. Because of our high complication rate with colon interposition in patients with wide-gap anomalies, we advocate expectant management with suctioning of the upper pouch and feeding through gastrostomy tubes while awaiting natural elongation of the esophagus. Delayed primary anastomosis can be performed between 8 and 12 weeks as advocated by Puri and associates [28] and Bensoussan and colleagues [29]. In cases of preexisting cervical esophagostomy, the technique advocated by Kimura and Soper [30] of extrathoracic esophageal elon-

6 Ann Thorac Surg TSAI ET AL 1997;64: ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA 783 gation seems promising and may offer an alternative to esophageal replacement. Although the morbidity associated with surgical repair of those anomalies is high, this does not affect the overall survival. The high complication rate associated with colon interposition suggests that one should preserve the native esophagus as a primary conduit whenever feasible. If one excludes infants with major associated lifethreatening congenital anomalies, survivals exceeding 90% can be achieved. References 1. Haight C, Towsley HA. Congenital atresia of the esophagus with tracheoesophageal fistula: extrapleural ligation of fistula and end-to-end anastomosis of esophageal segments. Surg Gynecol Obstet 1943;76: Poenaru D, Laberge JM, Neilson IR, Guttman FM. A new prognostic classification for esophageal atresia. Surgery 1993;113: Engum SA, Grosfeld JL, West KW, Rescorla FJ, Scherer TR. Analysis of morbidity and mortality in 227 cases of esophageal atresia and/or tracheoesophageal fistula over two decades. Arch Surg 1995;130: Redo SF. Congenital esophageal atresia and tracheoesophageal fistula fifteen year experience. NY State J Med 1975;75: Gross RE. The surgery of infancy and childhood. Philadelphia: Saunders, Waterston DJ, Bonham-Carter RE, Aberdeen E. Oesophageal atresia: tracheo-oesophageal fistula: a study of survival in 218 infants. Lancet 1962;1: Manning PB, Morgan RA, Coran AG, et al. Fifty years experience with esophageal atresia and tracheoesophageal fistula. Ann Surg 1986;204: McKinnon LJ, Kosloske AM. Prediction and prevention of anastomotic complications of esophageal atresia and tracheo-esophageal fistula. J Pediatr Surg 1990;25: Spitz L, Kiely E, Brereton RJ, Drake D. Management of esophageal atresia. World J Surg 1993;17: Holder TM, Cloud DT, Lewis JE, et al. Esophageal atresia and tracheoesophageal fistula: a survey of its members by the surgical section of the American Academy of Pediatrics. Pediatrics 1964;34: Bishop PJ, Klein MD, Phillipart AI, et al. Transpleural repair of esophageal atresia without a primary gastrostomy: 240 patients treated between 1951 and J Pediatr Surg 1985; 20: Touloukian RJ. Long-term results following repair of esophageal atresia by end-to-side anastomosis and ligation of the tracheoesophageal fistula. J Pediatr Surg 1981;16: Spitz L, Kiely E, Brereton RJ. Esophageal atresia: five year experience with 148 cases. J Pediatr Surg 1987;22: Sillen U, Hagberg S, Rubenson A, Werkmaster K. Management of esophageal atresia: review of 16 years experience. J Pediatr Surg 1988;23: Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ. Anastomotic leakage following surgery for esophageal atresia. J Pediatr Surg 1992;27: Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ. Anastomotic stricture following repair of esophageal atresia. J Pediatr Surg 1990;25: Louhimo I, Lindahl H. Esophageal atresia: primary results of 500 consecutively treated patients. J Pediatr Surg 1983;18: DeLorimer A, Harrison MR. Esophageal replacement. In: Aschraft KW, Holder TM, eds. Pediatric esophageal surgery. Orlando: Grune & Stratton, 1986: Holder TM, Ashcraft RW, Sharp RJ, Amoury RA. Care of infants with esophageal atresia, tracheoesophageal fistula, and associated anomalies. J Thorac Cardiovasc Surg 1987;94: Livaditis A. Esophageal atresia. A method of overbridging large segmental gaps. Z Kinderchir 1973;13: Evans M. Application of Collis gastroplasty to the management of esophageal atresia. J Pediatr Surg 1995;30: Spitz L. Esophageal atresia: past, present, and future. J Pediatr Surg 1996;31: Slany E, Holzki J, Holschneider AM, et al. Flaccid trachea in tracheo-oesophageal malformations. Z Kinderchir 1990;45: Rescorla FJ, West KW, Schere LR, Grosfeld JL. The complex nature of type A (long-gap) esophageal atresia. Surgery 1994; 116: Howard R, Myers NA. Esophageal atresia: a technique for elongating the upper pouch. Surgery 1965;58: Shafer AD, David TE. Suture fistula as a means of connecting upper and lower segments in esophageal atresia. J Pediatr Surg 1974;9: Hendren WH, Hale JR. Esophageal atresia treated by electromagnetic bougienage and subsequent repair. J Pediatr Surg 1976;11: Puri P, Ninan GK, Blake NS, Fitzgerald RJ, Guiney EJ, O Donnell B. Delayed primary anastomosis for esophageal atresia: 18 months to 11 years follow up. J Pediatr Surg 1992; 27: Bensoussan AL, Letourneau JN, Blanchard H. Cure de l atresie de l oesophage (de types 1 et 2). Anastomose primaire differee. Chir Pediatr 1983;24: Kimura K, Soper RT. Multistaged extrathoracic esophageal elongation for long gap esophageal atresia. J Pediatr Surg 1994;29: Ein SH, Shandling B, Heiss K. Pure esophageal atresia: outlook in the 1990s. J Pediatr Surg 1993;28: Boyle EM, Irwin ED, Foker JD. Primary repair of ultra-longgap esophageal atresia: results without a lengthening procedure. Ann Thorac Surg 1994;57: INVITED COMMENTARY Doctor Tsai and her coauthors have presented a 20-year experience with a relatively small series of infants and children with esophageal atresia and tracheoesophageal fistula (EA/TEF). They have reported on 81 patients of whom 68 underwent definitive treatment of their anomaly. Their overall survival rates are excellent, with favorable cases (Waterston risk groups A and B) achieving a survival between 89% and 100%. Although the Waterston classification has been traditionally used to categorize infants with EA/TEF, the criteria are really out of date in the 1990s; the new classification reported by Spitz and associates [1] in 1991 is more appropriate today. The only criteria of importance in this new classification are weight of more or less than 1,500 g and the presence or absence of severe cardiac anomalies. Although the survival rates in this series are comparable with those seen in other, current, large series, such as those reported by Spitz, Myers, and myself, the complication rates in this series are significantly higher. For instance, in a series of almost 600 patients at the Univer by The Society of Thoracic Surgeons /97/$17.00 Published by Elsevier Science Inc PII S (97)

Esophageal Atresia with Tracheoesophageal Fistula: Ten Years of Experience in an Institute

Esophageal Atresia with Tracheoesophageal Fistula: Ten Years of Experience in an Institute ORIGINAL ARTICLE Esophageal Atresia with Tracheoesophageal Fistula: Ten Years of Experience in an Institute Chia-Feng Yang, Wen-Jue Soong*, Mei-Jy Jeng, Shu-Jen Chen, Yu-Sheng Lee, Pei-Chen Tsao, Betau

More information

Physical Exam. Vitals stable on room air Abdomen soft, non-distented Normal external genitalia Patent anus No limb anomalies

Physical Exam. Vitals stable on room air Abdomen soft, non-distented Normal external genitalia Patent anus No limb anomalies Case Presentation 1 day-old full-term baby girl noted to have drooling of saliva and increased secretions at birth Fetal US @32wks had shown polyhydramnios Birth weight 3515g Apgar 7@1min and 8@5min Unable

More information

A simple technique of oblique anastomosis can prevent stricture formation in primary repair of esophageal atresia

A simple technique of oblique anastomosis can prevent stricture formation in primary repair of esophageal atresia Journal of Pediatric Surgery (2012) 47, 1767 1771 www.elsevier.com/locate/jpedsurg Operative Techniques A simple technique of oblique anastomosis can prevent stricture formation in primary repair of esophageal

More information

Mehmet Melek 1 and Ufuk Cobanoglu Introduction. 2. Material and Method

Mehmet Melek 1 and Ufuk Cobanoglu Introduction. 2. Material and Method Gastroenterology Research and Practice Volume 2011, Article ID 527323, 4 pages doi:10.1155/2011/527323 Methodology Report A New Technique in Primary Repair of Congenital Esophageal Atresia Preventing Anastomotic

More information

An 8-year experience of esophageal atresia repair in Sarvar children hospital (Mashhad- IRAN)

An 8-year experience of esophageal atresia repair in Sarvar children hospital (Mashhad- IRAN) An 8-year experience of esophageal atresia repair in Sarvar children hospital (Mashhad- IRAN) Mehran Hiradfar* Ahmad Bazrafshan* Marjan Judi** Mohammad Gharavi*** - Reza Shojaeian**** * Associate professor

More information

Lung Function Abnormalities Following Repaired Esophageal Atresia and Tracheoesophageal Fistula

Lung Function Abnormalities Following Repaired Esophageal Atresia and Tracheoesophageal Fistula Bahrain Medical Bulletin, Vol. 27, No.4, December 2005 Lung Function Abnormalities Following Repaired Esophageal Atresia and Tracheoesophageal Fistula Hanaa Banjar, MD, FRCPC* Introduction: Esophageal

More information

Esophageal Atresia and Tracheoesophageal Fistula

Esophageal Atresia and Tracheoesophageal Fistula Esophageal Atresia and Tracheoesophageal Fistula James V. Richardson, M.D., Sharon E. Heintz, P.A., Nicholas P. Rossi, M.D., Creighton B. Wright, M.D., Donald B. Doty, M.D., and Johann L. Ehrenhaft, M.D.

More information

Oesophageal atresia and associated anomalies

Oesophageal atresia and associated anomalies Archives of Disease in Childhood, 1989, 64, 364-368 Oesophageal atresia and associated anomalies S CHIrTMITRAPAP, L SPITZ, E M KIELY, AND R J BRERETON The Hospital for Sick Children, Great Ormond Street,

More information

POSTOPERATIVE CONGENITAL ESOPHAGEAL ATRESIA COMPLICATIONS: A REVIEW

POSTOPERATIVE CONGENITAL ESOPHAGEAL ATRESIA COMPLICATIONS: A REVIEW CHILDREN S HOSPITAL II POSTOPERATIVE CONGENITAL ESOPHAGEAL ATRESIA COMPLICATIONS: A REVIEW Dr. Nguyen Thuy Hanh Ngan Neonatal Department CONTENTS 1. Background 2. Classification 3. Management 4. Complications

More information

IN 1943, using an extrapleural approach, Haight and

IN 1943, using an extrapleural approach, Haight and Long-Term Analysis of Children With Esophageal Atresia and Tracheoesophageal Fistula D.C. Little, F.J. Rescorla, J.L. Grosfeld, K.W. West, L.R. Scherer, and S.A. Engum Indianapolis, Indiana Background/Purpose:

More information

An 18-year experience of tracheoesophageal fistula and esophageal atresia

An 18-year experience of tracheoesophageal fistula and esophageal atresia Original article DOI: 10.3345/kjp.2010.53.6.705 Korean J Pediatr 2010;53(6):705-710 An 18-year experience of tracheoesophageal fistula and esophageal atresia Juhee Seo, M.D. 2, Do Yeon Kim 2, Ai Rhan Kim,

More information

Impact of Suture Choice on Stricture Formation Following Repair of Esophageal Atresia

Impact of Suture Choice on Stricture Formation Following Repair of Esophageal Atresia Annals of Pediatric Surgery, Vol 3, No 2, April 2007 PP 75-79 Original Article Impact of Suture Choice on Stricture Formation Following Repair of Esophageal Atresia Shawn D. St. Peter, Patricia A. Valusek,

More information

Supplemental Information

Supplemental Information ARTICLE Supplemental Information SUPPLEMENTAL TABLE 6 Mosaic and Partial Trisomies Thirty-eight VLBW infants were identified with T13, of whom 2 had mosaic T13. T18 was reported for 128 infants, of whom

More information

Alyssa Brzenski MD May 2, 2012

Alyssa Brzenski MD May 2, 2012 Alyssa Brzenski MD May 2, 2012 Overview Background Pre repair bronchoscopy Thorascopic repair To extubate or not? Esophageal atresia treatment of long gap esophageal atresia Complications following TEF/EA

More information

Abstract Introduction We present the experiences from two European centers performing the

Abstract Introduction We present the experiences from two European centers performing the Original Article 3 The Foker Technique (FT) and Kimura Advancement (KA) for the Treatment of Children with Long-Gap Esophageal Atresia (LGEA): Lessons Learned at Two European Centers Mariusz Sroka 1 Robin

More information

Long Gap Esophageal Atresia and Esophageal Replacement: Moving Toward a Separation?

Long Gap Esophageal Atresia and Esophageal Replacement: Moving Toward a Separation? Long Gap Esophageal Atresia and Esophageal Replacement: Moving Toward a Separation? By P. Bagolan, B.D. Iacobelli, P. De Angelis, G. Federici di Abriola, R. Laviani, A. Trucchi, M. Orzalesi, and L. Dall

More information

Tracheoesophageal Fistula and Esophageal Atresia

Tracheoesophageal Fistula and Esophageal Atresia Patient and Family Education Tracheoesophageal Fistula and Esophageal Atresia What is tracheoesophageal fistula? The word fistula means abnormal connection. Tracheoesophageal fistula (TEF) is a condition

More information

Retrosternal ileocolic esophageal replacement in children revisited

Retrosternal ileocolic esophageal replacement in children revisited Retrosternal ileocolic esophageal replacement in children revisited Antirejfux role of the ileocecal valve The risk of postoperative reflux and pulmonary aspiration with straight colon or gastric tube

More information

ISSN East Cent. Afr. J. surg

ISSN East Cent. Afr. J. surg ISSN 073-9990 East Cent. Afr. J. surg Management of Congenital Tracheosophageal Atrasia and Fistula: A preliminary Bi-Cenre Study in Nigeria. E. Aiwanlehi., C. Odion, O. Osasumwen3 Department of Surgery,

More information

Absent Pulmonary Valve Syndrome

Absent Pulmonary Valve Syndrome Absent Pulmonary Valve Syndrome Fact sheet on Absent Pulmonary Valve Syndrome In this condition, which has some similarities to Fallot's Tetralogy, there is a VSD with narrowing at the pulmonary valve.

More information

Thoracoscopic repair of esophageal atresia with a distal fistula lessons from the first 10 operations

Thoracoscopic repair of esophageal atresia with a distal fistula lessons from the first 10 operations Original paper Thoracoscopic repair of esophageal atresia with a distal fistula lessons from the first 10 operations Paweł Nachulewicz 1, Kamila Zaborowska 1, Błażej Rogowski 1, Anita Kalińska 1, Marzena

More information

CONGENITAL HEART DISEASE (CHD)

CONGENITAL HEART DISEASE (CHD) CONGENITAL HEART DISEASE (CHD) DEFINITION It is the result of a structural or functional abnormality of the cardiovascular system at birth GENERAL FEATURES OF CHD Structural defects due to specific disturbance

More information

PBLD Anesthetic Management of Tracheoesophageal Fistula in a Premature Newborn with VACTERL Association and Hypoplastic Left Heart Syndrome

PBLD Anesthetic Management of Tracheoesophageal Fistula in a Premature Newborn with VACTERL Association and Hypoplastic Left Heart Syndrome PBLD Anesthetic Management of Tracheoesophageal Fistula in a Premature Newborn with VACTERL Association and Hypoplastic Left Heart Syndrome Moderators: Greg Latham, MD, Assistant Professor, Anesthesiology

More information

Congenital Heart Defects

Congenital Heart Defects Normal Heart Congenital Heart Defects 1. Patent Ductus Arteriosus The ductus arteriosus connects the main pulmonary artery to the aorta. In utero, it allows the blood leaving the right ventricle to bypass

More information

Long-gap Oesophageal Atresia

Long-gap Oesophageal Atresia Long-gap Atresia A guide for parents Nate (6 weeks old) Atresia Research Association Are there different types of OA? There are four main types of oesophageal atresia with or without TOF. These include

More information

Esophageal Perforation

Esophageal Perforation Esophageal Perforation Dr. Carmine Simone Thoracic Surgeon, Division of General Surgery Head, Division of Critical Care May 15, 2006 Overview Case presentation Radiology Pre-operative management Operative

More information

Departement of Surgery Faculty of Medicine University Sumatera Utara

Departement of Surgery Faculty of Medicine University Sumatera Utara SSS EESOPHAGEAL HPOSAGEAL DISORDERS IN SURGICAL PERSPECTIVE Departement of Surgery Faculty of Medicine University Sumatera Utara CONTENT 1. Esophageal Atresia 2. Achalasia 3. Esophageal Rupture 4. Tumor

More information

Original Article. Associated Anomalies and Clinical Outcomes in Infants with Omphalocele: A Single-centre 10-year Review

Original Article. Associated Anomalies and Clinical Outcomes in Infants with Omphalocele: A Single-centre 10-year Review HK J Paediatr (new series) 2018;23:220-224 Original Article Associated Anomalies and Clinical Outcomes in Infants with Omphalocele: A Single-centre 10-year Review YY CHEE, MSC WONG, RMS WONG, KY WONG,

More information

Dysphagia after EA repair. Disclosure. Learning objectives 9/17/2013

Dysphagia after EA repair. Disclosure. Learning objectives 9/17/2013 Dysphagia after EA repair Christophe Faure, M.D. Professor of Pediatrics, Division of Pediatric Gastroenterology, Sainte-Justine University Health Center, Université de Montréal, Montréal, QC, Canada christophe.faure@umontreal.ca

More information

Anatomy & Physiology

Anatomy & Physiology 1 Anatomy & Physiology Heart is divided into four chambers, two atrias & two ventricles. Atrioventricular valves (tricuspid & mitral) separate the atria from ventricles. they open & close to control flow

More information

Topics for discussion. Pediatric General Surgery. Physiology. Surgical Newborns. Neonatal Intestinal Obstruction

Topics for discussion. Pediatric General Surgery. Physiology. Surgical Newborns. Neonatal Intestinal Obstruction Topics for discussion Pediatric General Surgery Professor General & Thoracic Surgery What makes Pediatric Surgery unique? Neonatal intestinal obstruction Abdominal wall defects Inguinal hernias Appendicitis

More information

Tracheo-oesophageal fistula and oesophageal atresia:

Tracheo-oesophageal fistula and oesophageal atresia: Thorax (197), 25, 46. Tracheo-oesophageal fistula and oesophageal atresia: results of 3 years' experience MICHAEL P. HOLDEN and GEOFFREY H. WOOLER Department of Cardiothoracic Surgery, The General Infirmary

More information

The surgical approach to esophageal atresia repair and the management of long-gap atresia: results of a survey

The surgical approach to esophageal atresia repair and the management of long-gap atresia: results of a survey Seminars in Pediatric Surgery (2009) 18, 44-49 The surgical approach to esophageal atresia repair and the management of long-gap atresia: results of a survey Ori Ron, Paolo De Coppi, Agostino Pierro From

More information

The Combined Collis-Nissen Operation: Early Assessment of Reflwx Control

The Combined Collis-Nissen Operation: Early Assessment of Reflwx Control ORIGINAL ARTICLES The Combined Collis-Nissen Operation: Early Assessment of Reflwx Control Mark B. Orringer, M.D., and Jay S. Orringer, M.D. ABSTRACT This report summarizes the clinical experience with

More information

Gastric transposition in infants and children

Gastric transposition in infants and children DOI 10.1007/s00383-010-2736-9 REVIEW ARTICLE Gastric transposition in infants and children Robert A. Cowles Arnold G. Coran Accepted: 6 September 2010 Ó Springer-Verlag 2010 Abstract The loss of esophageal

More information

Neonatology ICD-10 documentation

Neonatology ICD-10 documentation Neonatology documentation Seven key impacts to documentation 1. Disease or disorder site 2. Acuity and/or encounter status of treatment 3. Etiology, causative agent, or disease type and injury/ poisoning

More information

Introduction. Study Design. Background. Operative Procedure-I

Introduction. Study Design. Background. Operative Procedure-I Risk Factors for Mortality After the Norwood Procedure Using Right Ventricle to Pulmonary Artery Shunt Ann Thorac Surg 2009;87:178 86 86 Addressor: R1 胡祐寧 2009/3/4 AM7:30 SICU 討論室 Introduction Hypoplastic

More information

SWISS SOCIETY OF NEONATOLOGY. Unusual cause of pneumoperitoneum in a spontaneously breathing 1-day-old term infant

SWISS SOCIETY OF NEONATOLOGY. Unusual cause of pneumoperitoneum in a spontaneously breathing 1-day-old term infant SWISS SOCIETY OF NEONATOLOGY Unusual cause of pneumoperitoneum in a spontaneously breathing 1-day-old term infant March 2012 2 Berger TM, Winiker H, Neonatal and Pediatric Intensive Care Unit (BTM), Pediatric

More information

Esophageal Replacement for Long-Gap Esophageal Atresia in a Resource-Limited Setting

Esophageal Replacement for Long-Gap Esophageal Atresia in a Resource-Limited Setting CASE REPORT Esophageal Replacement for Long-Gap Esophageal Atresia in a Resource-Limited Setting Saula PW, Kuremu RT School of Medicine, Moi University Correspondence to: Dr. Peter Walter Saula, P.O Box

More information

Transient malformations like PDA and PDA of prematurity were not considered. We have divided cardiac malformations in 2 groups:

Transient malformations like PDA and PDA of prematurity were not considered. We have divided cardiac malformations in 2 groups: CARDIAC MALFORMATIONS DETECTED AT BIRTH Anwar Dudin-MD, Annie Rambaud-Cousson-MD, Mahmoud Nashashibi-MD Pediatric Department Makassed Hospital Jerusalem Diagnosis of congenital heart disease in the neonatal

More information

AORTIC COARCTATION. Synonyms: - Coarctation of the aorta

AORTIC COARCTATION. Synonyms: - Coarctation of the aorta AORTIC COARCTATION Synonyms: - Coarctation of the aorta Definition: Aortic coarctation is a congenital narrowing of the aorta, usually located after the left subclavian artery, near the ductus or the ligamentum

More information

An Overview of Bronchopulmonary Dysplasia and Chronic Lung Disease in Infancy

An Overview of Bronchopulmonary Dysplasia and Chronic Lung Disease in Infancy An Overview of Bronchopulmonary Dysplasia and Chronic Lung Disease in Infancy Housekeeping: I have no financial disclosures Learning objectives: Develop an understanding of bronchopulmonary dysplasia (BPD)

More information

Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experience

Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experience Seminars in Pediatric Surgery (2009) 18, 12-19 Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experience Alan E. Mortell, FRCSI, MD, Richard G. Azizkhan, MD From the Division of

More information

Original Article. Double Aortic Arch in Infants and Children CH XIE, FQ GONG, GP JIANG, SL FU. Key words. Background

Original Article. Double Aortic Arch in Infants and Children CH XIE, FQ GONG, GP JIANG, SL FU. Key words. Background HK J Paediatr (new series) 2018;23:233-238 Original Article Double Aortic Arch in Infants and Children CH XIE, FQ GONG, GP JIANG, SL FU Abstract Key words Background: This study aimed to report the diagnosis,

More information

The application of autologous pulmonary artery in surgical correction of complicated aortic arch anomaly

The application of autologous pulmonary artery in surgical correction of complicated aortic arch anomaly Original Article The application of autologous pulmonary artery in surgical correction of complicated aortic arch anomaly Shusheng Wen, Jianzheng Cen, Jimei Chen, Gang Xu, Biaochuan He, Yun Teng, Jian

More information

Cardiac Catheterization Cases Primary Cardiac Diagnoses Facility 12 month period from to PRIMARY DIAGNOSES (one per patient)

Cardiac Catheterization Cases Primary Cardiac Diagnoses Facility 12 month period from to PRIMARY DIAGNOSES (one per patient) PRIMARY DIAGNOSES (one per patient) Septal Defects ASD (Atrial Septal Defect) PFO (Patent Foramen Ovale) ASD, Secundum ASD, Sinus venosus ASD, Coronary sinus ASD, Common atrium (single atrium) VSD (Ventricular

More information

Colon Patch Esophagoplasty: A Clinical Study For Chemical Burn Esophageal Stricture

Colon Patch Esophagoplasty: A Clinical Study For Chemical Burn Esophageal Stricture ISPUB.COM The Internet Journal of Surgery Volume 5 Number 1 Colon Patch Esophagoplasty: A Clinical Study For Chemical Burn Esophageal Stricture M Hourang, V Mehrabi Citation M Hourang, V Mehrabi. Colon

More information

Acquired pediatric esophageal diseases Imaging approaches and findings. M. Mearadji International Foundation for Pediatric Imaging Aid

Acquired pediatric esophageal diseases Imaging approaches and findings. M. Mearadji International Foundation for Pediatric Imaging Aid Acquired pediatric esophageal diseases Imaging approaches and findings M. Mearadji International Foundation for Pediatric Imaging Aid Acquired pediatric esophageal diseases The clinical signs of acquired

More information

Esophageal atresia B Lessons I have learned in a 40-year experience

Esophageal atresia B Lessons I have learned in a 40-year experience Journal of Pediatric Surgery (2006) 41, 1635 1640 www.elsevier.com/locate/jpedsurg Original articles Esophageal atresia B Lessons I have learned in a 40-year experience Lewis Spitz Emeritus Nuffield Professor

More information

Slide 1. Slide 2. Slide 3 CONGENITAL HEART DISEASE. Papworth Hospital NHS Trust INTRODUCTION. Jakub Kadlec/Catherine Sudarshan INTRODUCTION

Slide 1. Slide 2. Slide 3 CONGENITAL HEART DISEASE. Papworth Hospital NHS Trust INTRODUCTION. Jakub Kadlec/Catherine Sudarshan INTRODUCTION Slide 1 CONGENITAL HEART DISEASE Jakub Kadlec/Catherine Sudarshan NHS Trust Slide 2 INTRODUCTION Most common congenital illness in the newborn Affects about 4 9 / 1000 full-term live births in the UK 1.5

More information

Tetralogy of Fallot (TOF) with absent pulmonary valve

Tetralogy of Fallot (TOF) with absent pulmonary valve Repair of Tetralogy of Fallot with Absent Pulmonary Valve Syndrome Karl F. Welke, MD, and Ross M. Ungerleider, MD, MBA Tetralogy of Fallot (TOF) with absent pulmonary valve syndrome (APVS) occurs in 5%

More information

5.8 Congenital Heart Disease

5.8 Congenital Heart Disease 5.8 Congenital Heart Disease Congenital heart diseases (CHD) refer to structural or functional heart diseases, which are present at birth. Some of these lesions may be discovered later. prevalence of Chd

More information

FIG The inferior and posterior peritoneal reflection is easily

FIG The inferior and posterior peritoneal reflection is easily PSOAS HITCH, BOARI FLAP, AND COMBINATION OF PSOAS 7 HITCH AND BOARI FLAP The psoas hitch procedure, Boari flap, and transureteroureterostomy are useful operative procedures for reestablishing continuity

More information

PREMATURITY/ESTIMATED GESTATIONAL AGE

PREMATURITY/ESTIMATED GESTATIONAL AGE PREMATURITY/ESTIMATED GESTATIONAL AGE 765.21 < 24 completed weeks of gestation P07.21 Extreme immaturity of newborn, gestational age less than 23 completed weeks P07.22 Extreme immaturity of newborn, gestational

More information

Combined Collis-Nissen Reconstruction. of the esophagogastric junction at. Mark B. Orringer, M.D., and Herbert Sloan, M.D.

Combined Collis-Nissen Reconstruction. of the esophagogastric junction at. Mark B. Orringer, M.D., and Herbert Sloan, M.D. Combined Collis-Nissen Reconstruction of the Esophagogastric Junction Mark B. Orringer, M.D., and Herbert Sloan, M.D. ABSTRACT Recent reports have indicated that combined Collis-Belsey reconstruction of

More information

Pattern of Congenital Heart Disease A Hospital-Based Study *Sadiq Mohammed Al-Hamash MBChB, FICMS

Pattern of Congenital Heart Disease A Hospital-Based Study *Sadiq Mohammed Al-Hamash MBChB, FICMS Pattern of Congenital Heart Disease A Hospital-Based Study *Sadiq Mohammed Al-Hamash MBChB, FICMS ABSTRACT Background: The congenital heart disease occurs in 0,8% of live births and they have a wide spectrum

More information

Unusual presentations of late onset diaphragmatic hernia: a six year study

Unusual presentations of late onset diaphragmatic hernia: a six year study International Surgery Journal Aggarwal S et al. Int Surg J. 2017 Apr;4(4):1180-1184 http://www.ijsurgery.com pissn 2349-3305 eissn 2349-2902 Original Research Article DOI: http://dx.doi.org/10.18203/2349-2902.isj20170895

More information

SURGICAL TREATMENT AND OUTCOME OF CONGENITAL HEART DISEASE

SURGICAL TREATMENT AND OUTCOME OF CONGENITAL HEART DISEASE SURGICAL TREATMENT AND OUTCOME OF CONGENITAL HEART DISEASE Mr. W. Brawn Birmingham Children s Hospital. Aims of surgery The aim of surgery in congenital heart disease is to correct or palliate the heart

More information

LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: OPTIONS FOR SURGICAL MANAGEMENT

LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: OPTIONS FOR SURGICAL MANAGEMENT LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: OPTIONS FOR SURGICAL MANAGEMENT 10-13 March 2017 Ritz Carlton, Riyadh, Saudi Arabia Zohair AlHalees, MD Consultant, Cardiac Surgery Heart Centre LEFT VENTRICULAR

More information

ULTRASOUND OF THE FETAL HEART

ULTRASOUND OF THE FETAL HEART ULTRASOUND OF THE FETAL HEART Cameron A. Manbeian, MD Disclosure Statement Today s faculty: Cameron Manbeian, MD does not have any relevant financial relationships with commercial interests or affiliations

More information

In 1980, Bex and associates 1 first introduced the initial

In 1980, Bex and associates 1 first introduced the initial Technique of Aortic Translocation for the Management of Transposition of the Great Arteries with a Ventricular Septal Defect and Pulmonary Stenosis Victor O. Morell, MD, and Peter D. Wearden, MD, PhD In

More information

3/14/2011 MANAGEMENT OF NEWBORNS CARDIAC INTENSIVE CARE CONFERENCE FOR HEALTH PROFESSIONALS IRVINE, CA. MARCH 7, 2011 WITH HEART DEFECTS

3/14/2011 MANAGEMENT OF NEWBORNS CARDIAC INTENSIVE CARE CONFERENCE FOR HEALTH PROFESSIONALS IRVINE, CA. MARCH 7, 2011 WITH HEART DEFECTS CONFERENCE FOR HEALTH PROFESSIONALS IRVINE, CA. MARCH 7, 2011 MANAGEMENT OF NEWBORNS WITH HEART DEFECTS A NTHONY C. CHANG, MD, MBA, MPH M E D I C AL D I RE C T OR, HEART I N S T I T U T E C H I LDRE N

More information

The arterial switch operation has been the accepted procedure

The arterial switch operation has been the accepted procedure The Arterial Switch Procedure: Closed Coronary Artery Transfer Edward L. Bove, MD The arterial switch operation has been the accepted procedure for the repair of transposition of the great arteries (TGA)

More information

Tracheoesophageal fistula repair in a premature infant with VACTERL, infradiaphragmatic TAPVR, and pulmonary hypertension.

Tracheoesophageal fistula repair in a premature infant with VACTERL, infradiaphragmatic TAPVR, and pulmonary hypertension. PBLD Table # 26 Tracheoesophageal fistula repair in a premature infant with VACTERL, infradiaphragmatic TAPVR, and pulmonary hypertension. Moderators: Annette Schure, MD; Lauren Welsh, MD Objectives: 1.

More information

Deok Young Choi, Gil Hospital, Gachon University NEONATES WITH EBSTEIN S ANOMALY: PROBLEMS AND SOLUTION

Deok Young Choi, Gil Hospital, Gachon University NEONATES WITH EBSTEIN S ANOMALY: PROBLEMS AND SOLUTION Deok Young Choi, Gil Hospital, Gachon University NEONATES WITH EBSTEIN S ANOMALY: PROBLEMS AND SOLUTION Carpentier classification Chauvaud S, Carpentier A. Multimedia Manual of Cardiothoracic Surgery 2007

More information

Anorectal malformations include a wide spectrum of

Anorectal malformations include a wide spectrum of JOURNAL OF LAPAROENDOSCOPIC & ADVANCED SURGICAL TECHNIQUES Volume 20, Number 1, 2010 ª Mary Ann Liebert, Inc. DOI: 10.1089=lap.2008.0343 Laparoscopic-Assisted Pull-Through for Congenital Rectal Stenosis

More information

Coarctation of the aorta is a congenital narrowing of the

Coarctation of the aorta is a congenital narrowing of the Operative Risk Factors and Durability of Repair of Coarctation of the Aorta in the Neonate Walter H. Merrill, MD, Steven J. Hoff, MD, James R. Stewart, MD, Charles C. Elkins, MD, Thomas P. Graham, [r,

More information

Congenital Heart Disease in Radial Clubbed Hand

Congenital Heart Disease in Radial Clubbed Hand Archives of Disease in Childhood, 1971, 46, 345. Congenital Heart Disease in Radial Clubbed Hand Syndrome A. SIMCHA* From The Thoracic Unit, The Hospital for Sick Children, Great Ormond Street. London

More information

Notes: 1)Membranous part contribute in the formation of small portion in the septal cusp.

Notes: 1)Membranous part contribute in the formation of small portion in the septal cusp. Embryology 9 : Slide 16 : There is a sulcus between primitive ventricular and bulbis cordis that will disappear gradually and lead to the formation of one chamber which is called bulboventricular chamber.

More information

T ageal fistulas result from complications of mechanical

T ageal fistulas result from complications of mechanical Management of Acquired Nonmalignant Tracheoesophageal Fistula Douglas J. Mathisen, MD, Hermes C. Grillo, MD, John C. Wain, MD, and Alan D. Hilgenberg, MD Department of Surgery, Massachusetts General Hospital,

More information

THE TREATMENT OF OESOPHAGEAL ATRESIA*

THE TREATMENT OF OESOPHAGEAL ATRESIA* THE TREATMENT OF OESOPHAGEAL ATRESIA* Though both the anatomy and the symptomatology of the first cases of oesophageal atresia were described in detail more than 250 years ago, it was not until 1941 that

More information

Neonatal Airway Disorders, Treatments, and Outcomes. Steven Goudy, MD Pediatric Otolaryngology Emory University Medical Center

Neonatal Airway Disorders, Treatments, and Outcomes. Steven Goudy, MD Pediatric Otolaryngology Emory University Medical Center Neonatal Airway Disorders, Treatments, and Outcomes Steven Goudy, MD Pediatric Otolaryngology Emory University Medical Center Disclosure I have nothing to disclose Neonatal and Pediatric Tracheostomy Tracheostomy

More information

September 26, 2012 Philip Stockwell, MD Lifespan CVI Assistant Professor of Medicine (Clinical)

September 26, 2012 Philip Stockwell, MD Lifespan CVI Assistant Professor of Medicine (Clinical) September 26, 2012 Philip Stockwell, MD Lifespan CVI Assistant Professor of Medicine (Clinical) Advances in cardiac surgery have created a new population of adult patients with repaired congenital heart

More information

Accuracy of the Fetal Echocardiogram in Double-outlet Right Ventricle

Accuracy of the Fetal Echocardiogram in Double-outlet Right Ventricle Blackwell Publishing IncMalden, USACHDCongenital Heart Disease 2006 The Authors; Journal compilation 2006 Blackwell Publishing, Inc.? 200723237Original ArticleFetal Echocardiogram in Double-outlet Right

More information

Glossary of medical terms (grouped by affected system or organ)

Glossary of medical terms (grouped by affected system or organ) Glossary of medical terms (grouped by affected system or organ) Atrial septal defect (ASD) disorder of the heart that is present at birth involving a hole in the wall (septum) separating the two upper

More information

SWISS SOCIETY OF NEONATOLOGY. Neonatal gastric perforation

SWISS SOCIETY OF NEONATOLOGY. Neonatal gastric perforation SWISS SOCIETY OF NEONATOLOGY Neonatal gastric perforation September 2002 2 Zankl A, Stähelin J, Roth K, Boudny P and Zeilinger G, Children s Hospital of Aarau (ZA, SJ, RK, ZG) and Institute of Pathology

More information

Long-Gap Esophageal Atresia Gallo, Gabriele; Zwaveling, S.; Groen, Hendrik; Van der Zee, D.; Hulscher, Jan

Long-Gap Esophageal Atresia Gallo, Gabriele; Zwaveling, S.; Groen, Hendrik; Van der Zee, D.; Hulscher, Jan University of Groningen Long-Gap Esophageal Atresia Gallo, Gabriele; Zwaveling, S.; Groen, Hendrik; Van der Zee, D.; Hulscher, Jan Published in: European Journal of Pediatric Surgery DOI: 10.1055/s-0032-1331459

More information

THE RESULTS OF POSTERIOR SAGITTAL ANORECTOPLASTY IN ANORECTAL MALFORMATIONS

THE RESULTS OF POSTERIOR SAGITTAL ANORECTOPLASTY IN ANORECTAL MALFORMATIONS Arch Iranian Med 2005; 8 (4): 272 276 Original Article THE RESULTS OF POSTERIOR SAGITTAL ANORECTOPLASTY IN ANORECTAL MALFORMATIONS Ahmad Khaleghnejad-Tabari MD *, Mahmood Saeeda MD** Background: Posterior

More information

The first report of the Society of Thoracic Surgeons

The first report of the Society of Thoracic Surgeons REPORT The Society of Thoracic Surgeons National Congenital Heart Surgery Database Report: Analysis of the First Harvest (1994 1997) Constantine Mavroudis, MD, Melanie Gevitz, BA, W. Steves Ring, MD, Charles

More information

Common Defects With Expected Adult Survival:

Common Defects With Expected Adult Survival: Common Defects With Expected Adult Survival: Bicuspid aortic valve :Acyanotic Mitral valve prolapse Coarctation of aorta Pulmonary valve stenosis Atrial septal defect Patent ductus arteriosus (V.S.D.)

More information

Congenital Anomalies

Congenital Anomalies Congenital Anomalies Down Syndrome 7580 7580 DOWN''S SYNDROME Q900 Q90.0 : Trisomy 21, meiotic nondisjunction 7580 7580 DOWN''S SYNDROME Q901 Q90.1 : Trisomy 21, mosaicism (mitotic nondisjunction) 7580

More information

Gastroschisis Sequelae and Management

Gastroschisis Sequelae and Management Gastroschisis Sequelae and Management Mary Finn Gillian Lieberman, MD Primary Care Radiology Beth Israel Deaconess Medical Center Harvard Medical School April 2014 Outline I. Definition and Epidemiology

More information

Surgical Management Of TAPVR. Daniel A. Velez, M.D. Congenital Cardiac Surgeon Phoenix Children s Hospital

Surgical Management Of TAPVR. Daniel A. Velez, M.D. Congenital Cardiac Surgeon Phoenix Children s Hospital Surgical Management Of TAPVR Daniel A. Velez, M.D. Congenital Cardiac Surgeon Phoenix Children s Hospital No Disclosures Goals Review the embryology and anatomy Review Surgical Strategies for repair Discuss

More information

Surgical Treatment for Atrioventricular Septal Defect. Masakazu Nakao Consultant, Paediatric Cardiothoracic Surgery

Surgical Treatment for Atrioventricular Septal Defect. Masakazu Nakao Consultant, Paediatric Cardiothoracic Surgery Surgical Treatment for Atrioventricular Septal Defect Masakazu Nakao Consultant, Paediatric Cardiothoracic Surgery 1 History Rastelli classification (Rastelli) Pulmonary artery banding (Muller & Dammann)

More information

The pulmonary valve is the most common heart valve

The pulmonary valve is the most common heart valve Biologic versus Mechanical Valve Replacement of the Pulmonary Valve After Multiple Reconstructions of the RVOT Tract S. Adil Husain, MD, and John Brown, MD Indiana University School of Medicine, Department

More information

Adult Congenital Heart Disease: What All Echocardiographers Should Know Sharon L. Roble, MD, FACC Echo Hawaii 2016

Adult Congenital Heart Disease: What All Echocardiographers Should Know Sharon L. Roble, MD, FACC Echo Hawaii 2016 1 Adult Congenital Heart Disease: What All Echocardiographers Should Know Sharon L. Roble, MD, FACC Echo Hawaii 2016 DISCLOSURES I have no disclosures relevant to today s talk 2 Why should all echocardiographers

More information

MEDICAL SCIENCES Vol.I -Adult Congenital Heart Disease: A Challenging Population - Khalid Aly Sorour

MEDICAL SCIENCES Vol.I -Adult Congenital Heart Disease: A Challenging Population - Khalid Aly Sorour ADULT CONGENITAL HEART DISEASE: A CHALLENGING POPULATION Khalid Aly Sorour Cairo University, Kasr elaini Hospital, Egypt Keywords: Congenital heart disease, adult survival, specialized care centers. Contents

More information

Outcomes of Mitral Valve Repair for Mitral Regurgitation Due to Degenerative Disease

Outcomes of Mitral Valve Repair for Mitral Regurgitation Due to Degenerative Disease Outcomes of Mitral Valve Repair for Mitral Regurgitation Due to Degenerative Disease TIRONE E. DAVID, MD ; SEMIN THORAC CARDIOVASC SURG 19:116-120c 2007 ELSEVIER INC. PRESENTED BY INTERN 許士盟 Mitral valve

More information

PAEDIATRIC EMQs. Andrew A Mallick Paediatrics.info.

PAEDIATRIC EMQs. Andrew A Mallick Paediatrics.info. PAEDIATRIC EMQs Andrew A Mallick Paediatrics.info www.paediatrics.info Paediatric EMQs Paediatrics.info First published in the United Kingdom in 2012. While the advice and information in this book is believed

More information

Tracheobronchomalacia (TBM) is a rare congenital or

Tracheobronchomalacia (TBM) is a rare congenital or Surgical Treatment of Tracheomalacia by Bronchoscopic Monitored Aortopexy in Infants and Children Ulf Abdel-Rahman, MD, Peter Ahrens, Hans Gerd Fieguth, MD, Richard Kitz, Klaüs Heller, and Anton Moritz,

More information

Appendix A.1: Tier 1 Surgical Procedure Terms and Definitions

Appendix A.1: Tier 1 Surgical Procedure Terms and Definitions Appendix A.1: Tier 1 Surgical Procedure Terms and Definitions Tier 1 surgeries AV Canal Atrioventricular Septal Repair, Complete Repair of complete AV canal (AVSD) using one- or two-patch or other technique,

More information

The Chest X-ray for Cardiologists

The Chest X-ray for Cardiologists Mayo Clinic & British Cardiovascular Society at the Royal College of Physicians, London : 21-23-October 2013 Cases-Controversies-Updates 2013 The Chest X-ray for Cardiologists Michael Rubens Royal Brompton

More information

Coarctation of the aorta

Coarctation of the aorta T H E P E D I A T R I C C A R D I A C S U R G E R Y I N Q U E S T R E P O R T Coarctation of the aorta In the normal heart, blood flows to the body through the aorta, which connects to the left ventricle

More information

Chapter 124: Congenital Disorders of the Trachea. Bruce Benjamin

Chapter 124: Congenital Disorders of the Trachea. Bruce Benjamin Chapter 124: Congenital Disorders of the Trachea Bruce Benjamin Investigation of the larynx and pharynx may be incomplete in infants and children with congenital abnormalities without investigation of

More information

The first steps in the management of esophageal atresia

The first steps in the management of esophageal atresia 1 The first steps in the management of esophageal atresia AUTHORS Radhames E. Lizardo, MD a Stephanie M. Bedzis, MD b Marion C. Henry, MD, FACS a Romeo C. Ignacio, Jr., MD, FACS a a Department of Surgery,

More information

(i) Family 1. The male proband (1.III-1) from European descent was referred at

(i) Family 1. The male proband (1.III-1) from European descent was referred at 1 Supplementary Note Clinical descriptions of families (i) Family 1. The male proband (1.III-1) from European descent was referred at age 14 because of scoliosis. He had normal development. Physical evaluation

More information

RESULTS OF 102 CASES OF COMPLETE REPAIR OF CONGENITAL HEART DEFECTS IN PATIENTS WEIGHING 700 TO 2500 GRAMS

RESULTS OF 102 CASES OF COMPLETE REPAIR OF CONGENITAL HEART DEFECTS IN PATIENTS WEIGHING 700 TO 2500 GRAMS RESULTS OF 102 CASES OF COMPLETE REPAIR OF CONGENITAL HEART DEFECTS IN PATIENTS WEIGHING 700 TO 2500 GRAMS V. Mohan Reddy, MD a Doff B. McElhinney, MD a Theresa Sagrado, BA a Andrew J. Parry, MD a David

More information

Jejunum for bridging long-gap esophageal atresia

Jejunum for bridging long-gap esophageal atresia Seminars in Pediatric Surgery (2009) 18, 34-39 Jejunum for bridging long-gap esophageal atresia Klaas(N) M.A. Bax, MD, PhD, FRCS(Ed) From the Department of Pediatric Surgery, Erasmus MC-Sophia Children

More information

Tracheal stenosis in infants and children is typically characterized

Tracheal stenosis in infants and children is typically characterized Slide Tracheoplasty for Congenital Tracheal Stenosis Peter B. Manning, MD Tracheal stenosis in infants and children is typically characterized by the presence of complete cartilaginous tracheal rings and

More information

Free Esophageal Perforation Following Hybrid Visceral Debranching and Distal Endograft Extension to Repair a Ruptured Thoracoabdominal Aortic

Free Esophageal Perforation Following Hybrid Visceral Debranching and Distal Endograft Extension to Repair a Ruptured Thoracoabdominal Aortic Free Esophageal Perforation Following Hybrid Visceral Debranching and Distal Endograft Extension to Repair a Ruptured Thoracoabdominal Aortic Aneurysm History A 56-year-old gentleman, who had been referred

More information