Intestinal atresia and stenosis: analysis of survival in 120 cases

This report reviews the clinical presentation, operative management, and survival in 120 infants with intestinal atresia and stenosis treated from 1972 to 1984. Duodenal atresia occurred in 39 neonates and duodenal stenosis in 19. Thirty-two infants had severe associated anomalies. Operative management included duodenoduodenostomy in 47 infants, duodenotomy and web excision in four, and duodenojejunostomy in seven. Jejunoileal atresia occurred in 49 infants and stenosis in three. Six infants had cystic fibrosis and nine had gastroschisis. Operative therapy included wide proximal resection and end-to-end anastomosis in 18 infants, minimal resection with antimesenteric tapering enteroplasty and anastomosis in 14 neonates, and resection with temporary enterostomies in 20 infants. Twenty-nine infants (56%) required total parenteral nutrition. Colon atresia occurred in 11 infants and stenosis in one. Initial end-colostomy with subsequent resection and anastomosis was performed in 11 infants while one underwent a primary resection. The survival rate was 91% for duodenal defects, 87% for jejunoileal cases, and 100% for colonic anomalies. Death is currently caused by severe associated anomalies in infants with duodenal atresia and sepsis and total parenteral nutrition-related cholestasis with progressive liver failure in instances of jejunoileal atresia.     

Complications associated with surgical treatment of congenital intrinsic duodenal obstruction

Although survival rates for infants undergoing surgical treatment for congenital intrinsic duodenal obstruction are high, long-term follow-up suggests a high complication rate related to surgical therapy. We reviewed 33 neonates who underwent surgery for congenital intrinsic duodenal obstruction during the past 10 years. There were 20 girls and 13 boys; the mean gestational age was 36 weeks, and mean birthweight was 2,485 g. Bilious vomiting and intestinal obstruction were the most frequent presenting symptoms. Hydramnios was present in 75% of cases and 21% had associated Down's syndrome. Findings at laparotomy included duodenal atresia (14), annular pancreas (11), and duodenal diaphragm (8). The most frequent surgical procedure was side-to-side duodenoduodenostomy (DD), followed by duodenojejunostomy and resection of web with Heineke-Mikulicz type duodenoplasty. Bowel transit was reestablished at a mean of 13.1 days (range, 6 to 45 days). Seventy-percent of patients developed postoperative complications, the most frequent being megaduodenum with blind loop syndrome or bile reflux gastritis (22%), cholestatic jaundice (17%), gastroesophageal reflux (17%), delayed transit (8%), and bowel obstruction (8%). Six patients (18%) required secondary surgical procedures for postoperative complications (ie, megaduodenum, nonfunctioning anastomosis, missed intrinsic stenosis). Two patients died (6%). Stagnation and functional obstruction in the proximal duodenum is the main factor influencing the morbidity rate among these patients. Consideration should be given to the tapering duodenoplasty and diamond-shaped anastomosis in order to help reduce problems associated with megaduodenum and help restore earlier bowel transit.     

Primary repair of esophageal atresia without staging or preliminary gastrostomy.

Twenty-two neonates with esophageal atresia and a distal tracheoesophageal fistula were treated by primary repair. Only if an anastomotic leak was demonstrated was a tube gastrostomy for decompression with trangastric placement of a duodenal tube for feeding performed. Twelve newborns were classified as high-risk by the presence of prematurity with low birth weight (less than 2,250 gm), significant pneumonia, or obvious significant associated anomalies. There was one operative death and 1 late death. Three babies required a tube gastrotomy and duodenal tube for anastomotic leaks. All 20 survivors are symptom free and thriving. Progress in perioperative management of neonates with esophageal atresia may obviate the need for routine gastrostomy and for staging of high-risk newborns.     

Laparoscopic duodenoduodenostomy in the neonate

Background

Minimally invasive procedures are performed in neonates for an ever-expanding list of congenital anomalies. The laparoscopic repair of duodenal atresia and stenosis in the neonate is one such indication.

Method

We report our experience with the laparoscopic duodenoduodenostomy for duodenal atresia and stenosis in the neonate over the past 4 years. A retrospective chart review was conducted on all cases of duodenal atresia and stenosis diagnosed at our center between January 2004 and January 2008.

Results

Seventeen neonates underwent laparoscopic duodenoduodenostomy successfully during the period. Patient weight at surgery ranged from 1.35 to 3.75 kg. Most were operated on within the first week of life. Many had associated anomalies. There were no conversions to an open procedure, no intraoperative complications, and no anastomotic leaks observed. Time to full feeds averaged 12 days.

Conclusions

Laparoscopic duodenoduodenostomy in the neonate can be safely and successfully performed with excellent short-term outcome.     

Laparoscopic duodenal atresia repair using surgical U-clips: a novel technique

Laparoscopic repair of duodenal atresia has been reported. Reports to date have indicated use of standard laparoscopic suturing and knot tying. Unfortunately, there has been a high leak rate associated with the technique. We report our technique of using U-clips for the duodenoduodenostomy, thus limiting trauma to the duodenum during the anastomosis and less risk for postoperative leakage.     

The late nonfunctioning duodenal atresia repair

Since 1979, three newborn males had duodenoduodenostomies for duodenal atresia, and all babies had smooth uncomplicated postoperative courses. There were no other anomalies. Between 6 and 18 months postoperatively, each infant developed an obstruction at the anastomosis that was initially treated with prolonged nasogastric suction. Because conservative management did not relieve the obstruction, the first two babies were operated on several times with revision of the anastomosis and bypass procedures, all of which were slow to function and required prolonged intravenous alimentation. It then became apparent that the duodenoduodenostomy was functionally obstructed. Therefore, the third infant was successfully treated with plication only of the dilated atonic proximal duodenum. All three children are now thriving more than 2 years after their surgery.     

Duodenal atresia and stenosis: long-term follow-up over 30 years

Background

Duodenal atresia and stenosis is a frequent cause of congenital, intestinal obstruction. Current operative techniques and contemporary neonatal critical care result in a 5% morbidity and mortality rate, with late complications not uncommon, but unknown to short-term follow-up.

Methods

A retrospective review of patients with duodenal anomalies was performed from 1972 to 2001 at a tertiary, children’s hospital to identify late morbidity and mortality.

Results

Duodenal atresia or stenosis was identified in 169 patients. Twenty children required additional abdominal operations after their initial repair with average follow-up of 6 years (range, 1 month to 18 years) including fundoplication (13), operation for complicated peptic ulcer disease (4), and adhesiolysis (4). Sixteen children underwent revision of their initial repair: tapering duodenoplasty or duodenal plication (7), conversion of duodenojejunostomy to duodenoduodenostomy (3), redo duodenojejunostomy (3), redo duodenoduodenostomy (2), and conversion of gastrojejunostomy to duodenoduodenostomy (1). There were 10 late deaths (range, 3 months to 14 years) attributable to complex cardiac malformations (5), central nervous system bleeding (1), pneumonia (1), anastomotic leak (1), and multisystem organ failure (2).

Conclusions

Late complications occur in 12% of patients with congenital duodenal anomalies, and the associated late mortality rate is 6%, which is low but not negligible. Follow-up of these patients into adulthood is recommended to identify and address these late occurrences.     

Intestinal atresias: factors affecting clinical outcomes

Background

Despite improvements in care, intestinal atresias are associated with prolonged hospitalization and occasionally mortality. Although each type of atresia is distinct, it is unclear which factors impact clinical course. This study seeks to identify predictors of untoward outcome.

Methods

Neonates with duodenal, jejunal/ileal, and colonic atresia, treated at 1 institution from 1982 to 2005 were reviewed. Data were evaluated using nonparametric analysis of variance and logistic regression. Nonparametric data were expressed as medians with interquartile range (IQR).

Results

A total of 132 infants were evaluated including 63 with duodenal, 60 with jejunal/ileal, and 9 with colonic atresias. Overall mortality was 7% with associated congenital anomalies identified as an independent risk factor (P = .01). Infants with associated anomalies were more likely to have low birth weight (2.3 ± 0.8 vs 3.0 ± 0.8 kg, P = .01), which further increased mortality risk. Atresia location did not affect mortality or length of stay; however it did impact the time to full enteral nutrition with jejunal atresia requiring longer than duodenal (17 [IQR, 9-40 days] vs 10 [IQR, 7-70 days]; P = .01).

Conclusion

Overall mortality from intestinal atresia is low and is not dependent on the location of obstruction. Infants with birth weight less than 2 kg and associated anomalies are at an increased risk for prolonged hospital stay and mortality.     

Evaluation of the portal vein after duodenoduodenostomy for congenital duodenal stenosis associated with the preduodenal superior mesenteric vein, situs inversus, polysplenia, and malrotation

A male infant weighting 2970 g with total situs inversus, polysplenia, malrotation, duodenal stenosis, and complex cardiac anomalies, was admitted to our hospital. At 4 days of age, he underwent surgery that revealed a blood vessel passing over the duodenum from the mesenterium to the porta hepatis. A loose overbridging duodenoduodenostomy was performed to prevent compression of the vessel. The cardiac anomalies were corrected, and he could eat unrestricted diets. At the age of 1 year and 3 months, a 3-dimensional computed tomographic scan demonstrated that the vessel on the duodenum was the superior mesenteric vein (SMV), and it formed the portal vein with the splenic vein at the porta hepatis. Further, the scan revealed no compression of the SMV at the anastomosis. Doppler ultrasonography revealed a normal portal blood flow of 118.6 mL/min. This report describes the junction between the SMV and the splenic vein in a patient who had the SMV passing over the duodenum from the mesenterium. Correctly, patients previously diagnosed with a preduodenal portal vein could have a preduodenal SMV. The loose overbridging duodenoduodenostomy had advantages not only in passage of the anastomosis but also in maintenance of the portal blood flow for the congenital duodenal obstruction with the preduodenal SMV.     

Congenital intrinsic duodenal obstruction: problems in the diagnosis and management

Nineteen infants with intrinsic duodenal obstruction are analyzed. Atresia was the most common lesion. An exceptionally high rate of associated anomalies (73.7%) were present; Down's syndrome, the single most common anomaly, was seen in 47% of the infants. In seven infants, the diagnosis was delayed and in another three it was made intraoperatively while establishing a gastrostomy for esophageal atresia. Three infants died without operation because of gross prematurity and multiple anomalies. Of the 16 operated on, three died, one due to peritonitis and the other two because of metabolic derangements. Of the various operative procedures used, no significant difference was found in the final outcome of treatment. A schematic approach to the diagnosis and management is proposed.     

小儿腹腔镜十二指肠缝合吻合术治疗先天性十二指肠梗阻

目的评价腹腔镜辅助十二指肠缝合吻合术治疗小儿先天性十二指肠梗阻的效果。方法2003年2月～2008年7月,采用腹腔镜十二指肠缝合吻合术治疗13例先天性十二指肠梗阻,年龄2天～12岁（新生儿7例,婴幼儿3例,少儿3例）。十二指肠闭锁或狭窄7例,环状胰腺2例,肠系膜上动脉综合征（superior mesenteric artery syndrome,SMAS）3例,十二指肠前门静脉1例。结果除1例十二指肠前门静脉在小切口辅助下进行十二指肠空肠吻合术外,5例十二指肠隔膜在腹腔镜下纵行切开其前壁切除隔膜后完成横行吻合术,2例十二指肠闭锁和2例环状胰腺成功在腹腔镜下行十二指肠菱形吻合术,3例SMAS在腹腔镜下行十二指肠空肠Roux-en-Y吻合术。手术时间（97.0±18.2）min（75～180min）,无手术并发症出现。术后2～5d开始进食,7～9d恢复正常饮食。出院前上消化道造影检查吻合口通畅。11例获随访6个月～4年,其中8例随访〉1年,生长发育良好。结论腹腔镜十二指肠缝合吻合术效果可靠,可以在新生儿期安全实施,为治疗先天性十二指肠梗阻提供了一个良好的微创手术方式。     

腹腔镜下吻合术治疗新生儿十二指肠梗阻

目的探讨腹腔镜下吻合术治疗新生儿十二指肠梗阻(congenital duodenal obstruction,CDO)的疗效。方法回顾分析2008年12月～2011年9月53例CDO患者临床资料。腹腔镜组27例,Ⅱ型、Ⅲ型十二指肠闭锁及环状胰腺15例行十二指肠菱形吻合术,十二指肠狭窄及Ⅰ型十二指肠闭锁8例行十二指肠纵切横缝术,4例Ⅰ型十二指肠闭锁行十二指肠菱形吻合术;开腹组26例,以上3种术式分别为12例、8例、6例。对比2组手术时间、术后住院时间、术后进食时间、住院费用及术后并发症情况。结果与开腹组相比,腹腔镜组术后进食时间早[(5.8±1.6)d vs.(7.5±2.2)d,t=-3.060,P=0.004],但手术时间长[(113.8±39.2)min vs.(94.0±31.0)min,t=2.034,P=0.047]。2组术后住院时间和住院费用、并发症发生率差异无显著性(P>0.05)。结论腔镜下吻合术治疗新生儿十二指肠梗阻疗效肯定,在术后肠功能恢复上明显优于传统开腹手术。     

Early cavopulmonary anastomosis in very young infants after the Norwood procedure: impact on oxygenation, resource utilization, and mortality

BACKGROUND: The optimal timing of second-stage palliation after Norwood operations remains undefined. Advantages of early cavopulmonary anastomosis are early elimination of volume load and shortening the high-risk interstage period. Potential disadvantages include severe cyanosis, prolonged pleural drainage and hospitalization, and excess mortality. We reviewed our recent experience to evaluate the safety of early cavopulmonary anastomosis. METHODS: Eighty-five consecutive patients undergoing post-Norwood operation cavopulmonary anastomosis were divided into group I (cavopulmonary anastomosis at <4 months; n = 33) and group II (cavopulmonary anastomosis at >4 months; n = 52). Groups were compared for age; size; early and late mortality; preoperative, initial postoperative, and discharge oxygen saturation; and duration of mechanical ventilation, intensive care unit stay, pleural drainage, and hospitalization. RESULTS: Group I patients were younger than group II patients (94 +/- 21 days vs 165 +/- 44 days, respectively; P <.001) and smaller (4.8 +/- 0.8 kg vs 5.8 +/- 0.9 kg; P <.001). The preoperative oxygen saturation was not different (group I, 75% +/- 10%; group II, 78% +/- 8%; P =.142). The oxygen saturation was lower immediately after surgery in group I compared with group II (75% +/- 7% vs 81% +/- 7%, respectively; P <.001) but not by discharge (group I, 79% +/- 4%; group II, 80% +/- 4%). Younger patients were ventilated longer (62 +/- 86 hours vs 19 +/- 42 hours; P =.001), in the intensive care unit longer (130 +/- 111 hours vs 104 +/- 94 hours; P =.049), hospitalized longer (12.5 +/- 11.5 days vs 10.3 +/- 14.8 days; P =.012), and required longer pleural drainage (106 +/- 45 hours vs 104 +/- 93 hours; P =.046). Hospital survival was 100% in both groups. Actuarial survival to 12 months was 96% +/- 4% for group I and 96% +/- 3% for group II. CONCLUSIONS: Early cavopulmonary anastomosis after the Norwood operation is safe. Younger patients are more cyanotic initially after surgery and have a longer duration of mechanical ventilation, pleural drainage, intensive care unit stay, and hospitalization.     

Management of intestinal atresia in patients with gastroschisis

BACKGROUND/PURPOSE: Intestinal atresia occurs in approximately 10% to 20% of children with gastroschisis and may be missed at the initial closure if a thick peel obscures the bowel. Some investigators have identified intestinal atresia as a significant contributor to morbidity and mortality. The authors reviewed their experience with gastroschisis and intestinal atresia in an attempt to answer the following questions. What is the incidence of this association? How often is the intestinal atresia unrecognized as a result of the peel? What is the optimal management for infants with atresia and gastroschisis, and does the atresia affect morbidity or mortality? METHODS: The hospital charts and medical records of all patients with gastroschisis treated at our institution from 1969 to present were reviewed thoroughly. Parameters analyzed included gestational age (GA), birth weight (BW), antenatal diagnosis, mode of delivery, type of closure, era of repair, presence of other major anomalies, and development of necrotizing enterocolitis. Morbidity and mortality rates were examined. Characteristics of patients with and without atresia were compared. Chi-squared was used for crosstabular analysis. Sample parameters were compared with Student's t test. P values of less than.05 were considered significant. RESULTS: A total of 199 babies had gastroschisis and 25 (12.6%) had intestinal atresia. Intestinal atresia was initially unrecognized in 3 patients. Most patients (80%) underwent primary closure of the abdominal wall. Initial stoma formation and delayed anastomosis was performed in 12 (48%) patients, none of whom required prosthetic material for abdominal wall closure. Initial stomas were avoided in 5 patients who required SILASTIC (Dow Corning, Midland, MI) silos. Skin closure alone was used in 2 babies. The level of the atresia was most commonly jejunoileal (20 of 25, 80%). Mean hospital stay was increased in babies with intestinal atresia, 36.2 versus 63.1 days (P <.001). CONCLUSIONS: Although patients with intestinal atresia did have feeding delays, an increased incidence of adhesive intestinal obstruction, and prolonged hospitalization, neither chi(2) nor logistic regression analysis showed any correlation with mortality. Intestinal repair at the first operation is sometimes possible and depends on the severity of the peel. Delayed repair of the atresia after a period of bowel decompression and parenteral nutrition is preferred, but in certain situations (colonic atresia, necrotic intestine, complicated atresia) may not be possible. The combination of stomas and prosthetic material can be avoided in almost all patients. A management algorithm for patients with atresia and gastroschisis is discussed.     

Management of patients with combined tracheoesophageal fistula,esophageal atresia,and duodenal atresia

INTRODUCTION

Patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia (DA) pose a rare management challenge.

PRESENTATION OF CASE

Three patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia safely underwent a staged approach inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week. None of the patients suffered significant pre- or post-operative complications and our follow-up data (between 12 and 24 months) suggest that all patients eventually outgrow their reflux and respiratory symptoms.

DISCUSSION

While some authors support repair of all defects in one surgery, we recommend a staged approach. A gastrostomy tube is placed first for gastric decompression before TEF ligation and EA repair can be safely undertaken. The repair of the DA can then be performed within 3–7 days under controlled circumstances.

CONCLUSION

A staged approach of inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week resulted in excellent outcomes.     

Cardiac surgery in low birth weight infants: current outcomes

Low birth weight (LBW) is a risk factor for mortality in neonatal and infant heart surgery. The purpose of this study was to determine the contemporary outcomes and risk factors of cardiac surgery in low weight babies. The records of 75 consecutive infants weighing <2.5 kg having heart surgery were reviewed. The median weight was 2100 g (range 800-2500 g) and median age was 11 days (range 2-86 days). Half (n=38) of the infants were premature. Diagnoses included: arch obstruction (n=14), hypoplastic left heart syndrome (HLHS) (n=12), tetralogy of Fallot (ToF) or pulmonary atresia (PA)/ventricular septal defect (VSD) (n=11), transposition of the great arteries (TGA) (n=7), total anomalous pulmonary venous return (TAPVR) (n=5), and other (n=20). There were two early deaths. Follow-up was available on all infants with a median duration of 1320 days (range 6-3055 days). Cumulative Kaplan-Meier survival at one year was 90% [95% confidence interval (CI), 80-95%] and at five years was 88% (95% CI, 77-94%). Overall mortality amongst patients with genetic/chromosomal abnormalities was higher, 28% vs. 5.4% amongst patients without such abnormalities (P=0.008). Age, prematurity, preoperative mechanical ventilation, prostaglandins, non-cardiac organ dysfunction, extra-cardiac malformations, perioperative extracorporeal membrane oxygenation (ECMO), and type of procedure were not associated with significant differences in mortality. Cardiac surgery in LBW infants can be performed with low early and mid-term mortality. LBW infants with chromosomal/genetic anomalies have a higher risk.     

Annular pancreas in children: a recent decade's experience

Purpose

Annular pancreas is a recognized cause of duodenal obstruction in children. The authors sought to characterize the clinical, radiologic, and prognostic findings in this disorder through a 10-year review of all patients with annular pancreas.

Methods

A retrospective review of all annular pancreas cases identified between 1993 and 2002 at 2 tertiary pediatric surgical centers was completed. Factors analyzed included patient demographics, presenting symptoms, associated anomalies, radiologic findings, operation performed, postoperative outcomes, and complications.

Results

Sixteen patients were identified. Four patients (25%) were premature. Twelve patients (75%) presented during the first week of life and the remainder within the first year. All patients presented with vomiting, which was nonbilious in 15 (94%). Five patients (31 %) had chromosomal anomalies, and 6 (38%) had other major congenital malformations. Eleven patients (69%) presented with partial duodenal obstruction. Operations performed were duodenoduodenostomy in 14 (88%), duodenojejunostomy in 1 (6%), and Ladd’s procedure in 1 (6%). Enteral feedings were started, and full enteral feeding was achieved at an average of 8.4 days and 15.9 days after operation, respectively. All patients survived and were discharged after an average hospitalization of 24.0 days.

Conclusions

Patients with annular pancreas have preampullary duodenal obstruction, which is more commonly partial. Duodenoduodenostomy is the appropriate treatment. Prognosis is excellent, despite the frequent association with chromosomal anomalies and major congenital malformations.     

Tracheoesophageal anomalies in Waterston C neonates: a 30-year perspective

Two hundred and seventy-eight infants with congenital tracheoesophageal anomalies have been managed at the Columbus Children's Hospital since 1955. Fifty-four (20%) have been classified as high risk using Waterston's criteria of birth weight, associated anomalies, and pneumonitis. The infants have been divided into group I (n = 27, 1955 to 1969) and group II (n = 27, 1970 to 1984) to reflect the onset of mechanical ventilation and effective neonatal intensive care. The mean birth weight of infants in group II was significantly less than in group I (1,753 +/- 390 g v 1,950 +/- 505 g, P less than .05). The incidence of prematurity, measured by gestational age, has significantly increased with 9 of 27 (33%) infants in group II and 2 of 27 (7%) infants in group I less than 32 weeks gestation (P less than .05). The presence or severity of associated anomalies was not significantly different in groups I and II. Twenty patients in group I and 22 patients in group II underwent definitive management of their tracheoesophageal anomaly. Operative survival was 30% in group I and 74% in group II (P less than .05). Long-term survival was 15% in group I and 64% in group II (P less than .05). Early postoperative complications included aspiration pneumonitis, anastomotic leak, or stricture. The most frequent complications in long-term follow-up were recurrent pneumonias, which were observed in 9 of 16 children. Esophageal dysmotility or gastroesophageal reflux was documented in five of these children; four were managed successfully with positional or pharmacologic manipulations while one required an antireflux procedure. Thirty percent (5/16) are asymptomatic.(ABSTRACT TRUNCATED AT 250 WORDS)     

Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: a multi-institutional analysis

OBJECTIVES: For the past 60 years, successful repair of esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) has been performed via a thoracotomy. However, a number of reports have described adverse musculoskeletal sequelae following thoracotomy in infants and young children. Until now, only a few scattered case reports have detailed an individual surgeon's success with thoracoscopic repair of EA/TEF. This multi-institutional review represents the largest experience describing the results with this approach. METHODS: A cohort of international pediatric surgeons from centers that perform advanced laparoscopic and thoracoscopic operations in infants and children retrospectively reviewed their data on primary thoracoscopic repair in 104 newborns with EA/TEF. Newborns with EA without a distal TEF or those with an isolated TEF without EA were excluded. RESULTS: In these 104 patients, the mean age at operation was 1.2 days (+/-1.1), the mean weight was 2.6 kg (+/-0.5), the mean operative time was 129.9 minutes (+/-55.5), the mean days of mechanical ventilation were 3.6 (+/-5.8), and the mean days of total hospitalization were 18.1 (+/-18.6). Twelve (11.5%) infants developed an early leak or stricture at the anastomosis and 33 (31.7%) required esophageal dilatation at least once. Five operations (4.8%) were converted to an open thoracotomy and one was staged due to a long gap between the 2 esophageal segments. Twenty-five newborns (24.0%) later required a laparoscopic fundoplication. A recurrent fistula between the esophagus and trachea developed in 2 infants (1.9%). A number of other operations were required in these patients, including imperforate anus repair in 10 patients (7 high, 3 low), aortopexy (7), laparoscopic duodenal atresia repair (4), and various major cardiac operations (5). Three patients died, one related to the EA/TEF on the 20th postoperative day. CONCLUSIONS: The thoracoscopic repair of EA/TEF represents a natural evolution in the operative correction of this complicated congenital anomaly and can be safely performed by experienced endoscopic surgeons. The results presented are comparable to previous reports of babies undergoing repair through a thoracotomy. Based on the associated musculoskeletal problems following thoracotomy, there will likely be long-term benefits for babies with this anomaly undergoing the thoracoscopic repair.     

Total anomalous pulmonary venous connection: long-term appraisal with evolving technical solutions.

OBJECTIVES: To evaluate late outcome of non-isomeric total anomalous pulmonary venous connection (TAPVC) repair, controlling for anatomic subtypes and surgical technique. METHODS: Between 1983 and 2001, 89 patients (median age 54 days) underwent repair for supracardiac (38), cardiac (26), infracardiac (16) or mixed (nine) TAPVC. Ten patients (11.2%) presented associated anomalies other than PDA. Twenty-eight patients (31.5%) were emergencies, due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included the double-patch technique with left atrial enlargement in 29 patients and side-to-side anastomosis between the pulmonary venous (PV) confluence and the left atrium in 29 patients. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Total follow-up was 727.16 patient-years (mean 8.55 years, 98.8% complete). RESULTS: Early mortality was 7.86% (7/89). Ten patients (11.2%) underwent reintervention, including reoperation (eight), balloon dilation (one) and intraoperative stents placement (one), for anastomotic (four) or diffuse PV stenosis (six), with four late deaths. Kaplan-Meier survival is 87.3+/-0.036 SE% at 18.07 years with no difference according to anatomic type or surgical technique. Freedom from PV reintervention for operative survivors is 86.7+/-0.052 SE% at 18.07 years. Cox proportional hazard indicates associated anomalies (P=0.008) and reoperation for intrinsic PV stenosis (P=0.034) as independent predictors of mortality. According to logistic analysis, preoperative obstruction predicts higher risk of reintervention for intrinsic PV stenosis (P=0.022), while the double-patch technique increased the risk of late arrhythmias (P=0.005). CONCLUSIONS: Side-to-side anastomosis provides excellent results for TAPVC repair while left atrial enlargement procedures appear to be associated with higher risk of late arrhythmias. Although early and aggressive reintervention for recurrent PV obstruction is mandatory, intrinsic PV stenosis remains a predictor of adverse outcome.