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Repair of long gap esophageal atresia without anastomosis
Journal of Pediatric Surgery (2010) 45, 872–875
www.elsevier.com/locate/jpedsurg
Repair of long gap esophageal atresia without anastomosis Gustavo Stringel ⁎, Camelia Lawrence, Whitney McBride Division of Pediatric Surgery, Department of Surgery, Maria Fareri Children's Hospital, Westchester Medical Center, New York Medical College, Valhalla, NY 10595, USA Received 23 January 2010; accepted 2 February 2010
Key words: Esophageal atresia; Long gap; Suture fistula
Abstract Background/Purpose: Repair of long gap esophageal atresia represents a challenge. Several different techniques may be used. We describe 5 cases of long gap esophageal atresia managed successfully with suture approximation without anastomosis. Methods: Retrospective review identified 5 newborns (4 males; 1 female) with long gap esophageal atresia treated with suture approximation and subsequent endoscopic and fluoroscopic placement of string for guided dilatations. Three babies had esophageal atresia without fistula, and 2 had the common type with proximal atresia and distal tracheoesophageal fistula. The babies with pure esophageal atresia had delayed repair, and those with the common type had repair 2 days after birth. All had a gastrostomy for feedings. Results: All 5 babies recovered uneventfully. Three babies had spontaneous fistulization that allowed easy placement of guide wire and string. Two other babies required endoscopic and fluoroscopic combined fistula creation bypassing a long needle from the upper pouch to the lower one. Initially, all had string-guided dilatations that were subsequently converted to balloon dilatations. All babies had a functioning esophagus and did not need any further surgical intervention. An average of 8 postoperative dilatations were needed. Conclusions: The baby's own functional esophagus is superior to any esophageal replacement. Familiarity with different techniques to preserve it is therefore important. Suture approximation without anastomosis is a safe technique that can be applied to long gap esophageal atresia. The downside of this technique is a prolonged hospital stay, multiple dilatations, prolonged fasting, and therapy to learn to eat orally. © 2010 Elsevier Inc. All rights reserved.
Long gap esophageal atresia presents a challenge to pediatric surgeons. Many techniques have been described to correct this anomaly. However, no single technique is universally accepted or successful in all patients [1-7]. A suture fistula technique was initially described by Shafer and David [8] in 1974 and subsequently validated by Schullinger et al [2] and Marshall [9]. We describe 5
Presented at the 41st Annual Meeting of the Canadian Association of Paediatric Surgeons, Halifax, Nova Scotia, Canada, October 1-3, 2009. ⁎ Corresponding author. Westchester Medical Center, Valhalla, NY 10595, USA. Tel.: +1 914 493 7620; fax: +1 914 594 4933. E-mail address: [email protected] (G. Stringel). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.02.003
patients with long gap esophageal atresia managed successfully with this technique.
1. Methods 1.1. Patients We reviewed the cases of long gap esophageal atresia treated with the suture fistula technique at our institution during the last 15 years. There were 4 males and 1 female. Birth weights varied from 3.2 to 5.0 kg. Three babies had pure esophageal atresia, whereas 2 others had a proximal atresia with distal tracheoesophageal fistula. The 3 babies
Long gap esophageal atresia
873
with pure esophageal atresia had a delayed repair, 2 of which underwent surgery at 3 months of age and the third at 4 months of age. The 2 babies with proximal atresia and distal fistula had the repair at 2 days of age. The long gap was expected in the 3 babies with pure esophageal atresia. After extensive mobilization of both esophageal ends, it became obvious that an anastomosis could not be accomplished. We then contemplated all the options and decided to resort to the suture fistula technique. The 2 babies with the common type of esophageal atresia had an unexpected long gap, which did not allow for a primary anastomosis. They were successfully treated with suture fistula technique.
1.2. Surgical technique A standard extrapleural right thoracotomy was performed. The 2 esophageal ends were extensively mobilized, especially the upper pouch. The lower pouch had less mobilization to preserve the blood supply. The 2 babies with distal fistula had division and closure of the fistula and distal esophagus with fine nonabsorbable sutures. The pouches were not opened in the patients with pure esophageal atresia. The 2 ends were sutured together with significant tension. The technique of 2 nonabsorbable sutures was used as described by Marshall [9]. We used 2 stitches of 3-0 prolene (Ethicon, Somerville, NJ), ensuring that the 2 sutures were applied full thickness and through the lumen of both esophageal ends. In 2 babies, spontaneous fistulization did not occur. However, the 2 esophageal ends were together but without communication. One baby was managed by the pediatric
Fig. 1 Fistula created by interventional radiologist and pediatric surgeon under fluoroscopy.
Fig. 2 Rigid esophagoscope in the upper pouch and flexible scope in the lower pouch ready to create fistula under fluoroscopy.
surgeon (G. S.) and the interventional radiologist. A fistula was created under fluoroscopy bypassing a stiff guide wire from the lower pouch (via gastrostomy) to the upper pouch, which had a rigid infant esophagoscope to direct and retrieve the guide wire (Fig. 1). Subsequently, a silk thread (number 2) was passed through both pouches and used for subsequent string-guided dilatations.
Fig. 3 Rigid variceal injection needle passed from the upper pouch to the lower esophagus to advance glidewire.
874
G. Stringel et al. injection needle. The fistula was created by monitoring the passage of the needle with fluoroscopy and a flexible pediatric endoscope in the lower pouch via the gastrostomy. A size 0.018 in (0.46 mm)-diameter glidewire (Terumo Corporation, Somerset, NJ) was passed through the needle and retrieved with a snare by the flexible endoscope. A size 2-silk thread was placed for subsequent string-guided dilatations with Tucker dilators (Figs. 2-5). Spontaneous fistulization was demonstrated in 3 babies by contrast esophagram. A guide wire was passed by upper endoscopy and retrieved through the gastrostomy, and a string was placed for subsequent dilatations. The three babies with pure atresia had a gastrostomy at birth. The 2 babies with proximal esophageal atresia, and distal fistula had a gastrostomy at the same operative session after creation of the suture fistula.
2. Results
Fig. 4 Glidewire grasped with a snare using the flexible esophagoscope in the lower esophagus.
The second baby without spontaneous fistulization was treated bypassing a rigid infant esophagoscope into the upper pouch containing a rigid 22-gauge esophageal varices
Fig. 5
Glidewire in place across the esophageal anastomosis.
All babies recovered uneventfully. The string-guided esophageal dilatations started 2 weeks after passing the string. The initial 4 dilatations were done at 1-week intervals with bougies that gradually increased in size. The babies were subsequently converted to pneumatic balloon dilatations to avoid stretching the lower esophageal sphincter by the bougies. An average of 8 dilatations were needed to obtain an adequate lumen. Two babies required 3 more dilatations at 6 months and 1 year after discharge. The last patient treated 6 months ago is still receiving balloon esophageal dilatations once per month (Figs. 6 and 7).
Fig. 6
Balloon dilatation of fistula.
Long gap esophageal atresia
Fig. 7
Fistula dilated with esophageal balloon.
All patients had some degree of gastroesophageal reflux, but none has required antireflux surgery so far. None of the patients required further surgical intervention for management of the atresia. Follow-up varied from 6 months to 15 years. All patients have a functioning esophagus and are currently tolerating regular feedings.
3. Discussion The correction of long gap esophageal atresia requires pediatric surgeons to make choices. The definition of long gap is not universally accepted. Although some surgeons define it as more than 2 cm or 2 vertebral bodies [1], most textbooks agree that more than 3 cm is considered a long gap [10]. Some authors have noted that a long gap esophageal atresia is “in the eye of the beholder” [1]. Most pediatric surgeons agree that a gap of more than 5 cm is generally accepted as not amenable to correction with primary anastomosis. A popular definition of a long gap, albeit nonscientific, is the inability of an experienced pediatric surgeon to bring the 2 ends together. All of the babies described in the present report had a gap of more than 5 cm. Several techniques for achieving primary anastomosis in babies with long gap esophageal atresia have been described [1-10]. The authors of the present report have used many of these techniques during the past 30 years. No single
875 technique is effective in all patients. It is important for the pediatric surgeon to be familiar with different techniques that may apply to particular situations. There is general agreement that the baby's own esophagus is superior to any esophageal replacement. It therefore behooves the surgeon to try to save the baby's native esophagus when possible. The suture fistula technique is a reasonable alternative to preserve the esophagus. Most of the time, spontaneous fistulization will occur [2,3,8,9]. In cases without spontaneous fistulization, a second thoracotomy has been proposed by some authors to accomplish the esophageal anastomosis [3,9]. In our series, 2 cases did not fistulize. A communication between the 2 ends that were already apposed was created by an endoscopic approach under fluoroscopic control, thus avoiding a second thoracotomy. The suture fistula technique is safe, quick, technically easy, and allows the surgeon to bring the 2 esophageal ends together, even under considerable tension. A second thoracotomy and/or esophageal replacement surgery can be avoided by using this technique. The disadvantage of this method is the need for prolonged hospitalization, though some authors have proposed that these babies can be treated at home while awaiting esophageal anastomosis [11]. Other drawbacks include the need for multiple dilatations, the lack of oral feedings that may cause the baby to lose the ability to eat, and the need for occupational therapy to improve feeding and swallowing coordination.
References [1] Foker JE, Linden BC, Boyle Jr EM, et al. Development of a true primary repair for the full spectrum of esophageal atresia. Ann Surg 1997;226:533-43. [2] Schullinger JN, Vinocur CD, Santulli TV. The suture fistula technique in the repair of selected cases of esophageal atresia. J Pediatr Surg 1982;17:234-6. [3] Brown S, Nixon HH. Spontaneous anastomosis of the esophagus in esophageal atresia. J Pediatr Surg 1983;18:293-5. [4] Kimura K, Nishijima E, Tsugawa C, et al. A new approach for the salvage of unsuccessful esophageal atresia repair: a spiral myotomy and delayed definitive operation. J Pediatr Surg 1987;22:981-3. [5] Kimura K, Soper RT. Multistaged extrathoracic esophageal elongation for long gap esophageal atresia. J Pediatr Surg 1994;29:566-8. [6] Kimura K, Nishijima E, Tsugawa C, et al. Multistaged extrathoracic esophageal elongation procedure for long gap esophageal atresia: experience with 12 patients. J Pediatr Surg 2001;36:1725-7. [7] Al-Qahtani AR, Yazbeck S, Rosen NG, et al. Lengthening technique for long gap esophageal atresia and early anastomosis. J Pediatr Surg 2003;38:737-9. [8] Shafer AD, David TE. Suture fistula as a means of connecting upper and lower segments in esophageal atresia. J Pediatr Surg 1974;9:669-73. [9] Marshall DG. An alternative to an interposition procedure in esophageal atresia. J Pediatr Surg 1987;22:775-8. [10] Nakayama DK. Congenital abnormalities of the esophagus. In: O'Neill JA, Grosfeld JL, Fonkalsrud EW, Coran AG, Caldamone AA, editors. Principles of pediatric surgery, 2nd edSt Louis, MO: Mosby; 2003. p. 385-94. [11] Aziz D, Schiller D, Gerstle JT, et al. Can ‘long gap' esophageal atresia be safely managed at home while awaiting anastomosis? J Pediatr Surg 2003;38:705-8.
www.elsevier.com/locate/jpedsurg
Repair of long gap esophageal atresia without anastomosis Gustavo Stringel ⁎, Camelia Lawrence, Whitney McBride Division of Pediatric Surgery, Department of Surgery, Maria Fareri Children's Hospital, Westchester Medical Center, New York Medical College, Valhalla, NY 10595, USA Received 23 January 2010; accepted 2 February 2010
Key words: Esophageal atresia; Long gap; Suture fistula
Abstract Background/Purpose: Repair of long gap esophageal atresia represents a challenge. Several different techniques may be used. We describe 5 cases of long gap esophageal atresia managed successfully with suture approximation without anastomosis. Methods: Retrospective review identified 5 newborns (4 males; 1 female) with long gap esophageal atresia treated with suture approximation and subsequent endoscopic and fluoroscopic placement of string for guided dilatations. Three babies had esophageal atresia without fistula, and 2 had the common type with proximal atresia and distal tracheoesophageal fistula. The babies with pure esophageal atresia had delayed repair, and those with the common type had repair 2 days after birth. All had a gastrostomy for feedings. Results: All 5 babies recovered uneventfully. Three babies had spontaneous fistulization that allowed easy placement of guide wire and string. Two other babies required endoscopic and fluoroscopic combined fistula creation bypassing a long needle from the upper pouch to the lower one. Initially, all had string-guided dilatations that were subsequently converted to balloon dilatations. All babies had a functioning esophagus and did not need any further surgical intervention. An average of 8 postoperative dilatations were needed. Conclusions: The baby's own functional esophagus is superior to any esophageal replacement. Familiarity with different techniques to preserve it is therefore important. Suture approximation without anastomosis is a safe technique that can be applied to long gap esophageal atresia. The downside of this technique is a prolonged hospital stay, multiple dilatations, prolonged fasting, and therapy to learn to eat orally. © 2010 Elsevier Inc. All rights reserved.
Long gap esophageal atresia presents a challenge to pediatric surgeons. Many techniques have been described to correct this anomaly. However, no single technique is universally accepted or successful in all patients [1-7]. A suture fistula technique was initially described by Shafer and David [8] in 1974 and subsequently validated by Schullinger et al [2] and Marshall [9]. We describe 5
Presented at the 41st Annual Meeting of the Canadian Association of Paediatric Surgeons, Halifax, Nova Scotia, Canada, October 1-3, 2009. ⁎ Corresponding author. Westchester Medical Center, Valhalla, NY 10595, USA. Tel.: +1 914 493 7620; fax: +1 914 594 4933. E-mail address: [email protected] (G. Stringel). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.02.003
patients with long gap esophageal atresia managed successfully with this technique.
1. Methods 1.1. Patients We reviewed the cases of long gap esophageal atresia treated with the suture fistula technique at our institution during the last 15 years. There were 4 males and 1 female. Birth weights varied from 3.2 to 5.0 kg. Three babies had pure esophageal atresia, whereas 2 others had a proximal atresia with distal tracheoesophageal fistula. The 3 babies
Long gap esophageal atresia
873
with pure esophageal atresia had a delayed repair, 2 of which underwent surgery at 3 months of age and the third at 4 months of age. The 2 babies with proximal atresia and distal fistula had the repair at 2 days of age. The long gap was expected in the 3 babies with pure esophageal atresia. After extensive mobilization of both esophageal ends, it became obvious that an anastomosis could not be accomplished. We then contemplated all the options and decided to resort to the suture fistula technique. The 2 babies with the common type of esophageal atresia had an unexpected long gap, which did not allow for a primary anastomosis. They were successfully treated with suture fistula technique.
1.2. Surgical technique A standard extrapleural right thoracotomy was performed. The 2 esophageal ends were extensively mobilized, especially the upper pouch. The lower pouch had less mobilization to preserve the blood supply. The 2 babies with distal fistula had division and closure of the fistula and distal esophagus with fine nonabsorbable sutures. The pouches were not opened in the patients with pure esophageal atresia. The 2 ends were sutured together with significant tension. The technique of 2 nonabsorbable sutures was used as described by Marshall [9]. We used 2 stitches of 3-0 prolene (Ethicon, Somerville, NJ), ensuring that the 2 sutures were applied full thickness and through the lumen of both esophageal ends. In 2 babies, spontaneous fistulization did not occur. However, the 2 esophageal ends were together but without communication. One baby was managed by the pediatric
Fig. 1 Fistula created by interventional radiologist and pediatric surgeon under fluoroscopy.
Fig. 2 Rigid esophagoscope in the upper pouch and flexible scope in the lower pouch ready to create fistula under fluoroscopy.
surgeon (G. S.) and the interventional radiologist. A fistula was created under fluoroscopy bypassing a stiff guide wire from the lower pouch (via gastrostomy) to the upper pouch, which had a rigid infant esophagoscope to direct and retrieve the guide wire (Fig. 1). Subsequently, a silk thread (number 2) was passed through both pouches and used for subsequent string-guided dilatations.
Fig. 3 Rigid variceal injection needle passed from the upper pouch to the lower esophagus to advance glidewire.
874
G. Stringel et al. injection needle. The fistula was created by monitoring the passage of the needle with fluoroscopy and a flexible pediatric endoscope in the lower pouch via the gastrostomy. A size 0.018 in (0.46 mm)-diameter glidewire (Terumo Corporation, Somerset, NJ) was passed through the needle and retrieved with a snare by the flexible endoscope. A size 2-silk thread was placed for subsequent string-guided dilatations with Tucker dilators (Figs. 2-5). Spontaneous fistulization was demonstrated in 3 babies by contrast esophagram. A guide wire was passed by upper endoscopy and retrieved through the gastrostomy, and a string was placed for subsequent dilatations. The three babies with pure atresia had a gastrostomy at birth. The 2 babies with proximal esophageal atresia, and distal fistula had a gastrostomy at the same operative session after creation of the suture fistula.
2. Results
Fig. 4 Glidewire grasped with a snare using the flexible esophagoscope in the lower esophagus.
The second baby without spontaneous fistulization was treated bypassing a rigid infant esophagoscope into the upper pouch containing a rigid 22-gauge esophageal varices
Fig. 5
Glidewire in place across the esophageal anastomosis.
All babies recovered uneventfully. The string-guided esophageal dilatations started 2 weeks after passing the string. The initial 4 dilatations were done at 1-week intervals with bougies that gradually increased in size. The babies were subsequently converted to pneumatic balloon dilatations to avoid stretching the lower esophageal sphincter by the bougies. An average of 8 dilatations were needed to obtain an adequate lumen. Two babies required 3 more dilatations at 6 months and 1 year after discharge. The last patient treated 6 months ago is still receiving balloon esophageal dilatations once per month (Figs. 6 and 7).
Fig. 6
Balloon dilatation of fistula.
Long gap esophageal atresia
Fig. 7
Fistula dilated with esophageal balloon.
All patients had some degree of gastroesophageal reflux, but none has required antireflux surgery so far. None of the patients required further surgical intervention for management of the atresia. Follow-up varied from 6 months to 15 years. All patients have a functioning esophagus and are currently tolerating regular feedings.
3. Discussion The correction of long gap esophageal atresia requires pediatric surgeons to make choices. The definition of long gap is not universally accepted. Although some surgeons define it as more than 2 cm or 2 vertebral bodies [1], most textbooks agree that more than 3 cm is considered a long gap [10]. Some authors have noted that a long gap esophageal atresia is “in the eye of the beholder” [1]. Most pediatric surgeons agree that a gap of more than 5 cm is generally accepted as not amenable to correction with primary anastomosis. A popular definition of a long gap, albeit nonscientific, is the inability of an experienced pediatric surgeon to bring the 2 ends together. All of the babies described in the present report had a gap of more than 5 cm. Several techniques for achieving primary anastomosis in babies with long gap esophageal atresia have been described [1-10]. The authors of the present report have used many of these techniques during the past 30 years. No single
875 technique is effective in all patients. It is important for the pediatric surgeon to be familiar with different techniques that may apply to particular situations. There is general agreement that the baby's own esophagus is superior to any esophageal replacement. It therefore behooves the surgeon to try to save the baby's native esophagus when possible. The suture fistula technique is a reasonable alternative to preserve the esophagus. Most of the time, spontaneous fistulization will occur [2,3,8,9]. In cases without spontaneous fistulization, a second thoracotomy has been proposed by some authors to accomplish the esophageal anastomosis [3,9]. In our series, 2 cases did not fistulize. A communication between the 2 ends that were already apposed was created by an endoscopic approach under fluoroscopic control, thus avoiding a second thoracotomy. The suture fistula technique is safe, quick, technically easy, and allows the surgeon to bring the 2 esophageal ends together, even under considerable tension. A second thoracotomy and/or esophageal replacement surgery can be avoided by using this technique. The disadvantage of this method is the need for prolonged hospitalization, though some authors have proposed that these babies can be treated at home while awaiting esophageal anastomosis [11]. Other drawbacks include the need for multiple dilatations, the lack of oral feedings that may cause the baby to lose the ability to eat, and the need for occupational therapy to improve feeding and swallowing coordination.
References [1] Foker JE, Linden BC, Boyle Jr EM, et al. Development of a true primary repair for the full spectrum of esophageal atresia. Ann Surg 1997;226:533-43. [2] Schullinger JN, Vinocur CD, Santulli TV. The suture fistula technique in the repair of selected cases of esophageal atresia. J Pediatr Surg 1982;17:234-6. [3] Brown S, Nixon HH. Spontaneous anastomosis of the esophagus in esophageal atresia. J Pediatr Surg 1983;18:293-5. [4] Kimura K, Nishijima E, Tsugawa C, et al. A new approach for the salvage of unsuccessful esophageal atresia repair: a spiral myotomy and delayed definitive operation. J Pediatr Surg 1987;22:981-3. [5] Kimura K, Soper RT. Multistaged extrathoracic esophageal elongation for long gap esophageal atresia. J Pediatr Surg 1994;29:566-8. [6] Kimura K, Nishijima E, Tsugawa C, et al. Multistaged extrathoracic esophageal elongation procedure for long gap esophageal atresia: experience with 12 patients. J Pediatr Surg 2001;36:1725-7. [7] Al-Qahtani AR, Yazbeck S, Rosen NG, et al. Lengthening technique for long gap esophageal atresia and early anastomosis. J Pediatr Surg 2003;38:737-9. [8] Shafer AD, David TE. Suture fistula as a means of connecting upper and lower segments in esophageal atresia. J Pediatr Surg 1974;9:669-73. [9] Marshall DG. An alternative to an interposition procedure in esophageal atresia. J Pediatr Surg 1987;22:775-8. [10] Nakayama DK. Congenital abnormalities of the esophagus. In: O'Neill JA, Grosfeld JL, Fonkalsrud EW, Coran AG, Caldamone AA, editors. Principles of pediatric surgery, 2nd edSt Louis, MO: Mosby; 2003. p. 385-94. [11] Aziz D, Schiller D, Gerstle JT, et al. Can ‘long gap' esophageal atresia be safely managed at home while awaiting anastomosis? J Pediatr Surg 2003;38:705-8.