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The management of long gap esophageal atresia
Journal of Pediatric Surgery (2005) 40, 1542 – 1546
www.elsevier.com/locate/jpedsurg
The management of long gap esophageal atresia Emmanuelle Se´guier-Lipszyca, Amand Bonnarda, Sophie Aizenfiszb, Goharig Eneziana, Joel Maintenantc, Yves Aigraina, Pascal de Lagausied,* a
Department of Pediatric Surgery, Robert Debre´ Hospital, 75019 Paris, France Pediatric Intensive Care Unit, Robert Debre´ Hospital, 75019 Paris France c Department of Anaesthesiology, Robert Debre´ Hospital, 75019 Paris France d Department of Pediatric Surgery, Hoˆpital la Timone Enfant, 13005 Marseille, France b
Index words: Esophageal atresia; Long gap; Esocoloplasty; Esogastric disconnection
Abstract Background: Present management of esophageal atresia has enabled the survival rate to approach 95%. Controversy remains concerning the many options for the surgical management of long gap esophageal atresia without tracheoesophageal fistula and represents the difficulty of this pathology. In the last couple of years, we have had a nonexplained outbreak of cases of long gap esophageal atresia without tracheoesophageal fistula. This article reports our experience in the management of these children. Material and Methods: It is a retrospective study of all cases of long gap esophageal atresia without tracheoesophageal fistula managed in our institution since 1992, focusing on the antenatal period, delivery with weight and term, the associated malformations, the initial management, and the definitive surgery. Mann-Whitney U test was used for statistical analysis. Results: Ten cases (8.7%) of long gap esophageal atresia according to Ladd’s classification, 6 during the past 2 years, were taken in charge at Robert Debre´ Hospital between 1992 and 2002. There were 4 girls and 6 boys. Ten had a prenatal diagnosis of esophageal atresia. The average birth weight was 2496 g (range, 1400-3400 g) with an average term of 36.6-week gestation (range, 31.5-39.6). Delayed reconstruction was done in all children between 41 and 147 days of life (average of 102 days). Six had a direct anastomosis and 4 had a colonic esophagoplasty (3 with an esogastric disconnection during the same procedure). The average follow-up was 60 months (range, 27-133). There was 1 death owing to adenovirus infection at 5 years of age. Four children required a Nissen fundoplication for severe gastroesophageal reflux. At least, 2 children presented an anastomotic stricture which required pneumatic dilatations. Conclusion: Treatment options for long gap esophageal atresia generally require several stages over several months. We propose, for their management, a direct anastomosis at 4 months of age whenever it is possible. If not, we use a colonic esophagoplasty with an esogastric disconnection to control the gastroesophageal reflux which is responsible for strictures and respiratory impairment and does not obstruct the aperistaltic tube. D 2005 Elsevier Inc. All rights reserved.
T Corresponding author. Tel.: +33 04 91 38 66 73; fax: +33 04 91 38 47 14. E-mail address: [email protected] (P. de Lagausie). 0022-3468/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.06.007
Present management of esophageal atresia has enabled the survival rate to approach 95% [1]. Controversy remains concerning the many options for the surgical management
Clinical, operative, and postoperative data of the patients
Patient
Antenatal diagnosis
Neonatal management
Term (wk gestation)
Birth term (wk gestation)
Sex
Birth weight (g)
Associated anomalies
Surgery
1 2
21.4 31
37.6 31.5
F M
2500 2090
0 0
Gastrostomia/UPS Gastrostomia/UPS
3
36.5
37.5
F
2600
4
33
36
M
2330
Intestinal atresia + ARM + intestinal duplication + finger and facial anomalies Lumbar vertebral anomalies
5
31
35.6
M
2200
6
18
37
F
7
26
39.6
8
25
9
10 Average
Definitive surgery Date (d)
Other surgery
Follow-up (mo)
Status
90 117
Ok Esophageal dyskinesia Dead/ Adenovirus infection
Procedure
Postoperative complications
41 77
EA EA
0 GER
0 Nissen
Gastrostomia/UPS/ colostomia/intestinal resection
74
EA
GER/duodenal dilatation
Nissen/ duodenal modeling
71
Gastrostomia/UPS
82
EA
GER/esophageal stenosis
133
0
Gastrostomia/UPS
142
EA
3060
0
Gastrostomia/UPS
117
M
2350
Gastrostomia/UPS
110
0
0
33
Feeding difficulties Ok
38
M
3030
Cerebral, limbs, hip anomalies + hypospadias 0
GER
0
34
Ok
26
38.1
F
3400
0
Gastrostomia/ thoracotomy/cervical esophagostomia Gastrostomia/UPS
0
0
28
Ok
30 27.8
35.7 36.6
M
1400 2496
0
Gastrostomia/UPS
Colic esophagoplasty Colic esophagoplasty/ OGD Colic esophagoplasty/ OGD Colic esophagoplasty/ OGD EA
GER/esophageal stenosis Fistula
Nissen/ pneumatic dilatation Pneumatic dilatation 0
0
Nissen
27 60
Ok
92
135
147 102
33 32
Long gap esophageal atresia
Table 1
Dysphagia without stenosis Ok
M indicates male; F, female; ARM, anorectal malformation; UPS, upper pouch suction; EA, esophageal anastomosis; OGD, esogastric disconnection; GER, gastroesophageal reflux.
1543
1544 of long gap esophageal atresia without tracheoesophageal fistula and represents the difficulty of this pathology. All surgeons strive for a primary anastomosis in isolated long gap esophageal atresia cases. But when it is not possible, a delayed replacement procedure is necessary. All these surgical procedures are major undertakings and are associated with their own specific share of complications (leak, stricture, dilation, severe gastroesophageal reflux, etc) which are well described in literature. The pre- and postoperative pulmonary status appear essential in the management and the follow-up of these children, even if specific studies are almost nonexistent in literature. In the last couple of years, we have had a nonexplained outbreak of cases of long gap esophageal atresia without tracheoesophageal fistula. This article reports our experience and offers a suggestion in the management of these children with isolated long gap esophageal atresia.
1. Materials and methods We reviewed retrospectively all cases of long gap esophageal atresias without tracheoesophageal fistula managed in our institution between 1992 and 2002, focusing on the antenatal period, delivery with weight and term, the associated malformations, the initial management, and the definitive surgery. Immediate therapeutic approach consisted of performing a feeding gastrostomy on the first day of life with upper pouch suction. All children stayed in the intensive care surgical unit during the first postoperative days, with everyday chest physiotherapy, control of upper pouch suction every 6 hours and regular chest x-rays. Before definitive surgery, 1 contrast radiographic study per month was performed to try to detect the distance between the 2 esophageal pouches. We decided to perform the surgery in 2 cases: first, when the distance between the 2 esophageal pouches was about 3 vertebral bodies; second, 4 months after the initial procedure, whatever the distance, because we think that after, the growing of the pouches is nonexistent. After the definitive surgery, all patients were followed up on a regular basis and the initial results were obtained from the patients’ records. All patients had a contrast esophagogram with a chest x-ray, and those with respiratory symptoms, a pulmonary scan.
E. Se´guier-Lipszyc et al. There were 4 girls and 6 boys. Ten had a prenatal diagnosis of esophageal atresia (hydramnios; upper pouch with little gastric volume on prenatal ultrasonography or nonvisualized stomach) at 27.8-week gestation in average (range, 18-36.5). Three children had associated anomalies: 1 with an imperforate anus and multiple intestinal atresia; 1 with finger anomalies, hip subluxation, and cerebral anomalies; and 1 with lumbar vertebral anomalies. The average birth weight was 2496 g (range, 1400-3400 g) with an average term of 36.6-week gestation (range, 31.5-39.6). Forty percent are premature (V36 weeks gestation) in our series. Diagnosis was confirmed at birth. Two children had an additional surgical technique with gastrostomy (a cervical esophagostomy with a right thoracotomy for error of the estimated length of the distal pouch for the first and a laparotomy for associated anomalies for the second). Delayed reconstruction was done in all children at 102 days in average (range, 41-147). The average time at definitive surgery was significantly shorter in children operated on before 2001 (69 days, range 41-82), compared with those operated on after 2001 (124 days, range 92-147) ( P = .01). Six had a direct anastomosis and 4 had a colonic esophagoplasty (3 with an esogastric disconnection during the same time). One child had an anastomotic fistula 10 days after the surgery, which spontaneously disappeared. The average follow-up was 60 months (range, 27-133). There was 1 death owing to pulmonary adenovirus infection at 5 years of age (direct esophageal anastomosis). Four children required a Nissen fundoplication for severe gastroesophageal reflux. At least, 2 children presented an anastomotic stricture that required pneumatic dilatations. Total enteral nutrition has been obtained for all. Seven are force-feeding– weaned and 3 of those have a fractional oral feeding with night force-feeding. Eight children have a normal pulmonary examination. One girl has an abnormal chest x-ray and tomography 7 years after a direct anastomosis, requiring regular antibiotics owing to repeated infections, and 1 boy with a direct anastomosis and a Nissen fundoplication presents an abnormal chest x-ray and regular infections. The 3 children with the esophagogastric disconnection have no respiratory symptom. A satisfactory outcome was achieved in 90% of patients, with most progressing along the normal growth percentiles.
2. Results Between 1992 and 2002, a total of 116 infants with esophageal atresia were admitted to our institution. According to Ladd’s classification, 101 cases were type III, 1 was type II, 4 were type IV, and 10 (8.7%) were type I (long gap esophageal atresia without tracheoesophageal fistula). The study focused on these 10 cases: the first 4 cases have been managed before 2001 and the last 6 cases after 2001 (Table 1).
3. Discussion Concerning our 10 patients, 6 could have a direct anastomosis and 4 required a delayed reconstruction with a colonic esophagoplasty (and esogastric disconnection for 3). Surgical management and treatment of long gap esophageal atresia without tracheoesophageal fistula remains controversial. Preservation of the native esophagus is the surgeon’s priority because it represents the best conduit,
Long gap esophageal atresia even if its peristaltic activity is usually abnormal. Many different techniques have been described in literature to join the 2 esophageal pouches; none has proven to be adequate in all situations. Delayed repair requires a long hospitalization, but it enables the growth of the 2 esophageal stumps, either by external maneuvers that we do not use or, spontaneously, as shown by Puri et al [2], under the influence of the swallowing reflex for the upper pouch and of the gastroesophageal reflux for the lower pouch. Children whose esophagi are preserved seem to have a better outcome concerning swallowing function and symptoms related to gastroesophageal reflux [3]. We found no predictive factor that could tell which child will have direct anastomosis and that will require esophageal replacement. Regular radiologic series were disappointing because sometimes, the gap seemed important, but a direct anastomosis was possible and vice versa. Contrast radiographic studies are described insufficient because they cannot simulate the mobilization achieved with surgical dissection [3], and the gap is often a bit wider than what the x-ray study suggests [4]. In our series, the time at definitive procedure was significantly shorter for the children operated on before 2001 than for those operated on after. Despite this fact, there are more children in this last group who required an esophagoplasty. We prefer now waiting about 4 months to perform the definitive procedure. Several factors are found responsible for pulmonary impairment in these patients: repeated aspiration episodes despite permanent upper pouch suction, repermeation of an eventual fistula, and so forth. Thus, the only factor that made us perform surgery more or less early was the respiratory function and tolerance of the permanent suction of the proximal esophageal segment. Moreover, the pulmonary status of these children remains fragile and with high risk of aggravation after surgery (whether primary repair or delayed reconstruction). Pulmonary follow-up is necessary with a thoracic scan and respiratory function tests. Modern techniques of esophageal repair for atresia have rendered replacement an uncommon necessity. Elective ventilation protecting an anastomosis under tension, upper pouch flap operation [5,6], circular or spiral myotomy [7], full mobilization of the esophagus in the neck, and so forth make a direct end-to-end esophageal anastomosis possible most of the time after 12 weeks of age. Children whose esophagi are preserved seem to have better long-term outcome regarding swallowing function and gastroesophageal reflux – related symptoms [8]. When the waiting period is a minimum of 12 weeks and the child’s weight has at least doubled, delayed primary repair is successful in up to 75% of cases [4]. In our series, we have 60% of children with an esophageal anastomosis. Some argue that initial prolonged hospitalization is expensive, but it must be set against reduced long-term morbidity in these patients awaiting a normal life expectancy [9]. More recently, a home management has been proposed in selected situations with intermittent suction every 10 to 30 minutes when the child
1545 is awake in the presence of a nurse or well-trained parent with the same safety and efficiency than continuous upper pouch suction [10]. When a primary anastomosis is not possible, esophageal replacement becomes necessary. Many techniques exist for esophageal replacement: gastric transposition, interposition of the colon, the tubularized stomach [8], the reversed gastric tube [11], or the small intestine with a free [12] or pedicle graft [13,14]. Colonic interposition still represents one of the most commonly used techniques for esophageal replacement in children [15] and is the one we use in our institution. The long-term complications are limited and well described in articles concerning large series [16 -19]. It is performed in children younger than 1 year, but most authors agree that it should not, however, be performed in a newborn [16,20]. Most investigators agree that colon interposition serves mainly as a passive conduit regardless of the configuration (isoperistaltic, antiperistaltic, right, transverse, or descending colon) and empties by gravity [16]. When an acidreducing procedure is not performed simultaneously to the colon interposition, postoperative gastrocolic ulceration is not uncommon [15], but an antireflux procedure in such cases is difficult because it could obstruct the aperistaltic tube [4]. Reflux esophagitis was found in all esophageal remnants in a series of 8 pediatric patients who underwent esophageal replacement with a colon conduit [21]. Even in children with an esophageal anastomosis, Nissen fundoplication has been discussed because a complete wrap can lead to severe dysphagia in these patients with esophageal dysmotility [22]. Persistent gastroesophageal reflux limits the possibility of feeding orally these patients and often causes recurrent pulmonary infections with a risk of pulmonary function impairment. Thus we now perform simultaneously an esophagogastric disconnection to protect totally these children from gastroesophageal reflux. Bianchi [23] first described this technique in 1997. It is an important technique in nonverticalized children with a colonic neoesophagus, which functions with gravity. It is a reversible technique without any reflux, enabling preservation of the functional residual capacity of the lung [24]. This novel technique does not require multiple antireflux redos and protects the lungs with good pulmonary development, even in premature children without aspiration sequelae. It also enables progressive enteral feeding of these young children, first by gastrostomy, then orally. One year after surgery, 7 children eat normally with fractional oral feeding, and the gastrostomy is not used anymore in this population with normal growth curves. We continue to use the gastrostomy for night force-feeding only in 3 children below 1 SD for weight. For the moment, no child has been reconnected because our population is still very young, but it is feasible. Treatment options for long gap esophageal atresia generally require several stages over several months. We
1546 suggest, for their management, a direct anastomosis at 4 months of age whenever it is possible. If not, we use a colonic esophagoplasty with an esogastric disconnection to control the gastroesophageal reflux which is responsible for strictures and respiratory impairment and does not obstruct the aperistaltic tube.
References [1] Engum SA, Grosfeld JL, West KW, et al. Analysis of morbidity and mortality in 227 cases of oesophageal atresia and/or tracheoesophageal fistula over two decades. Arch Surg 1995;130:502 - 8. [2] Puri P, Blake N, O’Donnell B, et al. Delayed primary anastomosis following spontaneous growth of esophageal segments in esophageal atresia. J Pediatr Surg 1981;16:180 - 3. [3] Maksoud-Filho JG, Goncalves ME, Tannuri U, et al. An exclusively intraabdominal distal esophageal segment prevents primary delayed anastomosis in children with pure esophageal atresia. J Pediatr Surg 2002;37:1521 - 5. [4] Ein SH, Shandling B. Pure esophageal atresia: a 50-tear review. J Pediatr Surg 1994;29:1208 - 11. [5] Bar-Maor JA, Shoshany G, Sweed Y. Wide gap esophageal atresia: a new method to elongate the upper pouch. J Pediatr Surg 1989; 24:882 - 3. [6] Gough MH. Esophageal atresia: use of an anterior flap in the difficult anastomosis. J Pediatr Surg 1980;15:310 - 1. [7] Giacomoni MA, Tresoldi M, Zamana C, et al. Circular myotomy of the distal esophageal stump for long gap esophageal atresia. J Pediatr Surg 2001;36:855 - 7. [8] Puri P, Ninan GK, Blake NS, et al. Delayed primary anastomosis for esophageal atresia: 18 months’ to 11 years’ follow-up. J Pediatr Surg 1992;27:1127 - 30. [9] Pedersen JC, Klein RL, Andrews DA. Gastric tube as the primary procedure for pure esophageal atresia. J Pediatr Surg 1996;31:1233 - 5.
E. Se´guier-Lipszyc et al. [10] Aziz D, Schiller D, Gerstle JT, et al. Can dlong-gapT esophageal atresia be safely managed at home while awaiting anastomosis? J Pediatr Surg 2003;38:705 - 8. [11] Randolph JG. The reversed gastric tube for esophageal replacement in children. Pediatr Surg Int 1996;11:221 - 3. [12] Spicer RD, Cusick EL. Oesophageal substitution by jejunal free graft: follow-up data and an evaluation. Pediatr Surg Int 1996;11:227 - 9. [13] Bax NMA, Rfvekamp MH, Pull ter Gunne AJ, et al. Early one-stage orthotopic jejunal pedicle-graft interposition in long-gap esophageal atresia. Pediatr Surg Int 1994;9:483 - 5. [14] Saeki M, Tsuchida Y, Ogata T, et al. Long-term results of jejunal replacement of the esophagus. J Pediatr Surg 1988;23:483 - 9. [15] Ahmad SA, Sylvester KG, Hebra A, et al. Esophageal replacement using the colon: is it a good choice? J Pediatr Surg 1996;31:1026 - 30. [16] Hendren WH, Hendren WG. Colon interposition for the esophagus in children. J Pediatr Surg 1985;20:829 - 39. [17] German JC, Waterston DJ. Colon interposition for the replacement of the esophagus in children. J Pediatr Surg 1976;11:227 - 34. [18] Ure BM, Slany E, Eypasch EP, et al. Long-term functional results and quality of life after colon interposition of long-gap oesophageal atresia. Eur J Pediatr Surg 1995;5:206 - 10. [19] Lindahl H, Louhimo I, Virkola K. Colon interposition or gastric tube? Follow-up study of colon esophagus and gastric tube esophagus patients. J Pediatr Surg 1983;18:58 - 63. [20] Vargas Gomez M. Esophageal replacement in patients under 3 months of age. J Pediatr Surg 1994;29:487 - 91. [21] Shamberger RC, Eraklis AJ, Kozakewich HPW, et al. Fate of the distal esophageal remnant following esophageal replacement. J Pediatr Surg 1988;23:1210 - 4. [22] Wheatley MJ, Coran AG, Wesley JR. Efficacy of the Nissen fundoplication in the management of gastroesophageal reflux following esophageal atresia repair. J Pediatr Surg 1993;28:53 - 5. [23] Bianchi A. Total esophagogastric dissociation: an alternative approach. J Pediatr Surg 1997;32:1291 - 4. [24] de Lagausie P, Bonnard A, Schultz A, et al. Reflux in esophageal atresia, tracheo-oesophageal cleft and esophagocoloplasty: Bianchi’s procedure is an alternative approach. J Pediatr Surg 2005;40:666-9.
www.elsevier.com/locate/jpedsurg
The management of long gap esophageal atresia Emmanuelle Se´guier-Lipszyca, Amand Bonnarda, Sophie Aizenfiszb, Goharig Eneziana, Joel Maintenantc, Yves Aigraina, Pascal de Lagausied,* a
Department of Pediatric Surgery, Robert Debre´ Hospital, 75019 Paris, France Pediatric Intensive Care Unit, Robert Debre´ Hospital, 75019 Paris France c Department of Anaesthesiology, Robert Debre´ Hospital, 75019 Paris France d Department of Pediatric Surgery, Hoˆpital la Timone Enfant, 13005 Marseille, France b
Index words: Esophageal atresia; Long gap; Esocoloplasty; Esogastric disconnection
Abstract Background: Present management of esophageal atresia has enabled the survival rate to approach 95%. Controversy remains concerning the many options for the surgical management of long gap esophageal atresia without tracheoesophageal fistula and represents the difficulty of this pathology. In the last couple of years, we have had a nonexplained outbreak of cases of long gap esophageal atresia without tracheoesophageal fistula. This article reports our experience in the management of these children. Material and Methods: It is a retrospective study of all cases of long gap esophageal atresia without tracheoesophageal fistula managed in our institution since 1992, focusing on the antenatal period, delivery with weight and term, the associated malformations, the initial management, and the definitive surgery. Mann-Whitney U test was used for statistical analysis. Results: Ten cases (8.7%) of long gap esophageal atresia according to Ladd’s classification, 6 during the past 2 years, were taken in charge at Robert Debre´ Hospital between 1992 and 2002. There were 4 girls and 6 boys. Ten had a prenatal diagnosis of esophageal atresia. The average birth weight was 2496 g (range, 1400-3400 g) with an average term of 36.6-week gestation (range, 31.5-39.6). Delayed reconstruction was done in all children between 41 and 147 days of life (average of 102 days). Six had a direct anastomosis and 4 had a colonic esophagoplasty (3 with an esogastric disconnection during the same procedure). The average follow-up was 60 months (range, 27-133). There was 1 death owing to adenovirus infection at 5 years of age. Four children required a Nissen fundoplication for severe gastroesophageal reflux. At least, 2 children presented an anastomotic stricture which required pneumatic dilatations. Conclusion: Treatment options for long gap esophageal atresia generally require several stages over several months. We propose, for their management, a direct anastomosis at 4 months of age whenever it is possible. If not, we use a colonic esophagoplasty with an esogastric disconnection to control the gastroesophageal reflux which is responsible for strictures and respiratory impairment and does not obstruct the aperistaltic tube. D 2005 Elsevier Inc. All rights reserved.
T Corresponding author. Tel.: +33 04 91 38 66 73; fax: +33 04 91 38 47 14. E-mail address: [email protected] (P. de Lagausie). 0022-3468/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.06.007
Present management of esophageal atresia has enabled the survival rate to approach 95% [1]. Controversy remains concerning the many options for the surgical management
Clinical, operative, and postoperative data of the patients
Patient
Antenatal diagnosis
Neonatal management
Term (wk gestation)
Birth term (wk gestation)
Sex
Birth weight (g)
Associated anomalies
Surgery
1 2
21.4 31
37.6 31.5
F M
2500 2090
0 0
Gastrostomia/UPS Gastrostomia/UPS
3
36.5
37.5
F
2600
4
33
36
M
2330
Intestinal atresia + ARM + intestinal duplication + finger and facial anomalies Lumbar vertebral anomalies
5
31
35.6
M
2200
6
18
37
F
7
26
39.6
8
25
9
10 Average
Definitive surgery Date (d)
Other surgery
Follow-up (mo)
Status
90 117
Ok Esophageal dyskinesia Dead/ Adenovirus infection
Procedure
Postoperative complications
41 77
EA EA
0 GER
0 Nissen
Gastrostomia/UPS/ colostomia/intestinal resection
74
EA
GER/duodenal dilatation
Nissen/ duodenal modeling
71
Gastrostomia/UPS
82
EA
GER/esophageal stenosis
133
0
Gastrostomia/UPS
142
EA
3060
0
Gastrostomia/UPS
117
M
2350
Gastrostomia/UPS
110
0
0
33
Feeding difficulties Ok
38
M
3030
Cerebral, limbs, hip anomalies + hypospadias 0
GER
0
34
Ok
26
38.1
F
3400
0
Gastrostomia/ thoracotomy/cervical esophagostomia Gastrostomia/UPS
0
0
28
Ok
30 27.8
35.7 36.6
M
1400 2496
0
Gastrostomia/UPS
Colic esophagoplasty Colic esophagoplasty/ OGD Colic esophagoplasty/ OGD Colic esophagoplasty/ OGD EA
GER/esophageal stenosis Fistula
Nissen/ pneumatic dilatation Pneumatic dilatation 0
0
Nissen
27 60
Ok
92
135
147 102
33 32
Long gap esophageal atresia
Table 1
Dysphagia without stenosis Ok
M indicates male; F, female; ARM, anorectal malformation; UPS, upper pouch suction; EA, esophageal anastomosis; OGD, esogastric disconnection; GER, gastroesophageal reflux.
1543
1544 of long gap esophageal atresia without tracheoesophageal fistula and represents the difficulty of this pathology. All surgeons strive for a primary anastomosis in isolated long gap esophageal atresia cases. But when it is not possible, a delayed replacement procedure is necessary. All these surgical procedures are major undertakings and are associated with their own specific share of complications (leak, stricture, dilation, severe gastroesophageal reflux, etc) which are well described in literature. The pre- and postoperative pulmonary status appear essential in the management and the follow-up of these children, even if specific studies are almost nonexistent in literature. In the last couple of years, we have had a nonexplained outbreak of cases of long gap esophageal atresia without tracheoesophageal fistula. This article reports our experience and offers a suggestion in the management of these children with isolated long gap esophageal atresia.
1. Materials and methods We reviewed retrospectively all cases of long gap esophageal atresias without tracheoesophageal fistula managed in our institution between 1992 and 2002, focusing on the antenatal period, delivery with weight and term, the associated malformations, the initial management, and the definitive surgery. Immediate therapeutic approach consisted of performing a feeding gastrostomy on the first day of life with upper pouch suction. All children stayed in the intensive care surgical unit during the first postoperative days, with everyday chest physiotherapy, control of upper pouch suction every 6 hours and regular chest x-rays. Before definitive surgery, 1 contrast radiographic study per month was performed to try to detect the distance between the 2 esophageal pouches. We decided to perform the surgery in 2 cases: first, when the distance between the 2 esophageal pouches was about 3 vertebral bodies; second, 4 months after the initial procedure, whatever the distance, because we think that after, the growing of the pouches is nonexistent. After the definitive surgery, all patients were followed up on a regular basis and the initial results were obtained from the patients’ records. All patients had a contrast esophagogram with a chest x-ray, and those with respiratory symptoms, a pulmonary scan.
E. Se´guier-Lipszyc et al. There were 4 girls and 6 boys. Ten had a prenatal diagnosis of esophageal atresia (hydramnios; upper pouch with little gastric volume on prenatal ultrasonography or nonvisualized stomach) at 27.8-week gestation in average (range, 18-36.5). Three children had associated anomalies: 1 with an imperforate anus and multiple intestinal atresia; 1 with finger anomalies, hip subluxation, and cerebral anomalies; and 1 with lumbar vertebral anomalies. The average birth weight was 2496 g (range, 1400-3400 g) with an average term of 36.6-week gestation (range, 31.5-39.6). Forty percent are premature (V36 weeks gestation) in our series. Diagnosis was confirmed at birth. Two children had an additional surgical technique with gastrostomy (a cervical esophagostomy with a right thoracotomy for error of the estimated length of the distal pouch for the first and a laparotomy for associated anomalies for the second). Delayed reconstruction was done in all children at 102 days in average (range, 41-147). The average time at definitive surgery was significantly shorter in children operated on before 2001 (69 days, range 41-82), compared with those operated on after 2001 (124 days, range 92-147) ( P = .01). Six had a direct anastomosis and 4 had a colonic esophagoplasty (3 with an esogastric disconnection during the same time). One child had an anastomotic fistula 10 days after the surgery, which spontaneously disappeared. The average follow-up was 60 months (range, 27-133). There was 1 death owing to pulmonary adenovirus infection at 5 years of age (direct esophageal anastomosis). Four children required a Nissen fundoplication for severe gastroesophageal reflux. At least, 2 children presented an anastomotic stricture that required pneumatic dilatations. Total enteral nutrition has been obtained for all. Seven are force-feeding– weaned and 3 of those have a fractional oral feeding with night force-feeding. Eight children have a normal pulmonary examination. One girl has an abnormal chest x-ray and tomography 7 years after a direct anastomosis, requiring regular antibiotics owing to repeated infections, and 1 boy with a direct anastomosis and a Nissen fundoplication presents an abnormal chest x-ray and regular infections. The 3 children with the esophagogastric disconnection have no respiratory symptom. A satisfactory outcome was achieved in 90% of patients, with most progressing along the normal growth percentiles.
2. Results Between 1992 and 2002, a total of 116 infants with esophageal atresia were admitted to our institution. According to Ladd’s classification, 101 cases were type III, 1 was type II, 4 were type IV, and 10 (8.7%) were type I (long gap esophageal atresia without tracheoesophageal fistula). The study focused on these 10 cases: the first 4 cases have been managed before 2001 and the last 6 cases after 2001 (Table 1).
3. Discussion Concerning our 10 patients, 6 could have a direct anastomosis and 4 required a delayed reconstruction with a colonic esophagoplasty (and esogastric disconnection for 3). Surgical management and treatment of long gap esophageal atresia without tracheoesophageal fistula remains controversial. Preservation of the native esophagus is the surgeon’s priority because it represents the best conduit,
Long gap esophageal atresia even if its peristaltic activity is usually abnormal. Many different techniques have been described in literature to join the 2 esophageal pouches; none has proven to be adequate in all situations. Delayed repair requires a long hospitalization, but it enables the growth of the 2 esophageal stumps, either by external maneuvers that we do not use or, spontaneously, as shown by Puri et al [2], under the influence of the swallowing reflex for the upper pouch and of the gastroesophageal reflux for the lower pouch. Children whose esophagi are preserved seem to have a better outcome concerning swallowing function and symptoms related to gastroesophageal reflux [3]. We found no predictive factor that could tell which child will have direct anastomosis and that will require esophageal replacement. Regular radiologic series were disappointing because sometimes, the gap seemed important, but a direct anastomosis was possible and vice versa. Contrast radiographic studies are described insufficient because they cannot simulate the mobilization achieved with surgical dissection [3], and the gap is often a bit wider than what the x-ray study suggests [4]. In our series, the time at definitive procedure was significantly shorter for the children operated on before 2001 than for those operated on after. Despite this fact, there are more children in this last group who required an esophagoplasty. We prefer now waiting about 4 months to perform the definitive procedure. Several factors are found responsible for pulmonary impairment in these patients: repeated aspiration episodes despite permanent upper pouch suction, repermeation of an eventual fistula, and so forth. Thus, the only factor that made us perform surgery more or less early was the respiratory function and tolerance of the permanent suction of the proximal esophageal segment. Moreover, the pulmonary status of these children remains fragile and with high risk of aggravation after surgery (whether primary repair or delayed reconstruction). Pulmonary follow-up is necessary with a thoracic scan and respiratory function tests. Modern techniques of esophageal repair for atresia have rendered replacement an uncommon necessity. Elective ventilation protecting an anastomosis under tension, upper pouch flap operation [5,6], circular or spiral myotomy [7], full mobilization of the esophagus in the neck, and so forth make a direct end-to-end esophageal anastomosis possible most of the time after 12 weeks of age. Children whose esophagi are preserved seem to have better long-term outcome regarding swallowing function and gastroesophageal reflux – related symptoms [8]. When the waiting period is a minimum of 12 weeks and the child’s weight has at least doubled, delayed primary repair is successful in up to 75% of cases [4]. In our series, we have 60% of children with an esophageal anastomosis. Some argue that initial prolonged hospitalization is expensive, but it must be set against reduced long-term morbidity in these patients awaiting a normal life expectancy [9]. More recently, a home management has been proposed in selected situations with intermittent suction every 10 to 30 minutes when the child
1545 is awake in the presence of a nurse or well-trained parent with the same safety and efficiency than continuous upper pouch suction [10]. When a primary anastomosis is not possible, esophageal replacement becomes necessary. Many techniques exist for esophageal replacement: gastric transposition, interposition of the colon, the tubularized stomach [8], the reversed gastric tube [11], or the small intestine with a free [12] or pedicle graft [13,14]. Colonic interposition still represents one of the most commonly used techniques for esophageal replacement in children [15] and is the one we use in our institution. The long-term complications are limited and well described in articles concerning large series [16 -19]. It is performed in children younger than 1 year, but most authors agree that it should not, however, be performed in a newborn [16,20]. Most investigators agree that colon interposition serves mainly as a passive conduit regardless of the configuration (isoperistaltic, antiperistaltic, right, transverse, or descending colon) and empties by gravity [16]. When an acidreducing procedure is not performed simultaneously to the colon interposition, postoperative gastrocolic ulceration is not uncommon [15], but an antireflux procedure in such cases is difficult because it could obstruct the aperistaltic tube [4]. Reflux esophagitis was found in all esophageal remnants in a series of 8 pediatric patients who underwent esophageal replacement with a colon conduit [21]. Even in children with an esophageal anastomosis, Nissen fundoplication has been discussed because a complete wrap can lead to severe dysphagia in these patients with esophageal dysmotility [22]. Persistent gastroesophageal reflux limits the possibility of feeding orally these patients and often causes recurrent pulmonary infections with a risk of pulmonary function impairment. Thus we now perform simultaneously an esophagogastric disconnection to protect totally these children from gastroesophageal reflux. Bianchi [23] first described this technique in 1997. It is an important technique in nonverticalized children with a colonic neoesophagus, which functions with gravity. It is a reversible technique without any reflux, enabling preservation of the functional residual capacity of the lung [24]. This novel technique does not require multiple antireflux redos and protects the lungs with good pulmonary development, even in premature children without aspiration sequelae. It also enables progressive enteral feeding of these young children, first by gastrostomy, then orally. One year after surgery, 7 children eat normally with fractional oral feeding, and the gastrostomy is not used anymore in this population with normal growth curves. We continue to use the gastrostomy for night force-feeding only in 3 children below 1 SD for weight. For the moment, no child has been reconnected because our population is still very young, but it is feasible. Treatment options for long gap esophageal atresia generally require several stages over several months. We
1546 suggest, for their management, a direct anastomosis at 4 months of age whenever it is possible. If not, we use a colonic esophagoplasty with an esogastric disconnection to control the gastroesophageal reflux which is responsible for strictures and respiratory impairment and does not obstruct the aperistaltic tube.
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