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Thoracoscopic approach for congenital esophageal stenosis
Journal of Pediatric Surgery (2006) 41, E5 – E7
www.elsevier.com/locate/jpedsurg
Thoracoscopic approach for congenital esophageal stenosis Marcelo Martinez-Ferro*, Martin Rubio, Lisandro Piaggio, Pablo Laje Department of Pediatric Surgery, J.P. Garrahan National Children’s Hospital, Buenos Aires, Argentina Index words: Congenital esophageal stenosis; Esophageal dilation; Thoracoscopy
Abstract Congenital esophageal stenosis (CES) is an infrequent entity; however, many cases have been reported during the last years. Its incidence falls between 1 per 25,000 and 1 per 50,000 live births and is associated with other congenital malformations in 17% to 33% of cases (mainly esophageal atresia). Congenital esophageal stenosis is defined as an intrinsic alteration of the esophageal wall given by the presence of ectopic tracheobronchial tissue, membranous diaphragm, muscular hypertrophy, or diffuse fibrosis of the submucosa, among other causes. The therapeutic options include endoscopic dilation and resection plus anastomosis (by either laparotomy or thoracotomy, depending on the level of the stenosis). We present the case of a 1-month-old baby boy with a CES located in the distal esophagus that is associated with anophthalmia and micropenis. We treated the lesion by means of a thoracoscopic resection of the affected segment and an esophageal end-to-end anastomosis. The patient’s long-term outcome was uneventful. As far as we know, this is the first report on thoracoscopic resolution of a CES. D 2006 Elsevier Inc. All rights reserved.
Although congenital esophageal stenosis (CES) is an infrequent entity, many cases have been reported during the last years. Its incidence falls between 1 per 25,000 and 1 per 50,000 live births and is associated with other congenital malformations in approximately 17% to 33% of cases (mainly esophageal atresia [EA]) [1,2]. The preoperative diagnosis is sometimes difficult, and the surgical approach is always controversial [3]. We report on the case of a CES successfully treated by means of a thoracoscopic approach.
1. Case report A 1-month-old baby boy was admitted at our institution because of severe aspirative pneumonia. He was an ex 36-weeker diagnosed with bilateral anophthalmia and micro* Corresponding author. Tel.: +54 11 4790 5709; fax: +54 11 4702 3116. E-mail address: [email protected] (M. Martinez-Ferro). 0022-3468/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2006.06.022
penis. He had had respiratory symptoms during and after feedings and had been fed through a nasogastric tube for the last weeks before his admission. Plain abdominal x-rays showed a big gastric chamber. In an upper gastrointestinal contrast study, a severe stenosis of the distal esophagus was found, 3 cm in length, with a normal esophageal anatomy both proximal and distal to the lesion (Fig. 1).
1.1. Surgical technique After an unsuccessful attempt to dilate the stenosis with an 8-mm pneumatic balloon, we decided on surgical correction. The patient was placed in the left lateroventral decubitus position. We used 3 ports: a 4-mm port was placed in the 5th intercostal (IC) space/midaxillary line for the 4-mm 308 scope (Karl Storz 28731 BWA, Tuttlingen, Germany); a 5-mm port was placed high in the armpit area; and a 3-mm port was placed in the 8th IC space/posterior axillary line. A 5-mm Hg CO2 pneumothorax was established. Short (20 cm) 3-mm instruments were used
E6
M. Martinez-Ferro et al.
Fig. 3 Microscopic picture (hematoxylin and eosin) of the resected stenotic esophagus. Significant muscular hypertrophy and submucosal fibrosis of the esophageal wall are observed. Fig. 1 Initial barium swallow study. A long stenotic segment is observed in the distal esophagus.
to dissect the esophagus free. No clear evidence of the stricture location could be externally observed. At that moment, an endoesophageal balloon (3-Fr Fogarty balloon catheter) was introduced through the mouth by the anesthesiologist. The balloon was inflated distally and proximally until the stenotic area was clearly identified. We then concluded the esophagus dissection by sparing both vagus nerves and resecting the stricture (Fig. 2). An end-toend esophageal anastomosis was carried out using 5/0 PDS extracorporeal knots. A nasogastric tube was placed before finishing the anastomosis to feed the baby soon after the
surgery. A 12-Fr chest tube was placed through the inferior port site.
1.2. Follow-up Six days after the procedure, an esophagogram showed no leak and good progression of the contrast through the anastomotic site. We pulled the chest tube out and started feeding the baby. The pathology report was consistent with muscular hypertrophy and submucosal fibrosis of the esophageal wall (Fig. 3). One month after surgery, we dilated a slight stenosis of the anastomotic site with a 12mm-long pneumatic balloon. Two months after surgery, the contrast study findings were normal. Four months after surgery, although the anatomy and clearance were normal, severe gastroesophageal reflux (GER) was diagnosed by means of a barium contrast study. The 24-hour pH-probe study findings were abnormal as well. Antireflux measures and medication were started, but a laparoscopic Nissen fundoplication was performed on the baby when he was 6 months old. The patient’s recovery was uneventful, and he was discharged 6 days after surgery. The long-term followup is normal in terms of growth and nutrition.
2. Discussion
Fig. 2 Thoracoscopic view of the dissected esophagus. A balloon (B) has been inflated inside the esophageal lumen. Cephalic traction of the balloon delimitates the inferior aspect of the stenosis (Sten). Diaph indicates diaphragm; Vert, vertebrae.
Congenital esophageal stenosis is defined as the presence of an intrinsic esophageal stenosis caused by an abnormality in the structure of the esophageal wall [4,5]. The stenosis may or may not be symptomatic from the first days after birth. Most cases have been described in the lower esophagus [6], but some authors have reported higher lesions and multiple lesions in a single esophagus [7]. The stenosis may be caused by ectopic tracheobronchial tissue, membranous webs, hypertrophy of the muscular layer, or fibrosis of the submucosa [8].
Thoracoscopic approach for congenital esophageal stenosis Because most cases of pediatric esophageal stenosis are secondary to the repair of EAs or a complication of GER, these and other causes of secondary esophageal stenosis must be ruled out (eg, extrinsic compressions, foreign bodies) [9]. Those cases of CES associated with EA usually have tracheobronchial remnants in the esophageal wall [10]. Once the diagnosis of CES is confirmed, other abnormalities that may be related to it must also be searched for (eg, cardiac and intestinal malformations, chromosomal anomalies). We first attempted to treat our patient following the guidelines of Shorter et al [11] (ie, by means of dilations). However, because tracheobronchial ectopic remnants tend to be difficult to stretch, we failed and so decided to treat the patient surgically. Some authors have reported successful dilations in up to 42.9% of cases, but the results may vary depending on the histologic findings. In the case of esophageal webs, endoscopic and open perforation-dilation procedures have been described [12]. Kawahara et al [13] reported a high incidence of GER in patients with CES, probably caused by loss of the natural high-pressure zone after surgery. This was the case in our patient. As far as we know, this is the first report on thoracoscopic resolution of a CES. We think that it is a safe, effective, and reproducible technique.
References [1] Olguner M, Ozdemir T, Akgur F, et al. Congenital esophageal stenosis owing to tracheobronchial remnants: a case report. J Pediatr Surg 1997;32:1485 - 7.
E7 [2] Vasudevan S, Kerendi F, Lee H, et al. Management of congenital esophageal stenosis. J Pediatr Surg 2002;37:1024 - 6. [3] Takamizawa S, Tsugawa C, Mouri N, et al. Congenital esophageal stenosis: therapeutic strategy based on etiology. J Pediatr Surg 2002; 37:197 - 201. [4] Amae S, Nio M, Kamiyama T, et al. Clinical characteristics and management of congenital esophageal stenosis: a report on 14 cases. J Pediatr Surg 2003;38:565 - 70. [5] Ramesh J, Ramanujam T, Jayaram G. Congenital esophageal stenosis: report of three cases, literature review, and a proposed classification. Pediatr Surg Int 2001;17:188 - 92. [6] Karaguzel G, Tanyel C, Akcoren Z, et al. Intramural tracheobronchial remnants associated with esophageal atresia: diagnostic aids. Pediatr Surg Int 1993;8:138 - 9. [7] Grabowski T, Andrews DA. Upper esophageal stenosis: two case reports. J Pediatr Surg 1996;31:1438 - 9. [8] Murphy G, Yazbeck S, Russo P. Isolated congenital esophageal stenosis. J Pediatr Surg 1995;30:1238 - 41. [9] Neilson R, Croitoru P, Guttman F, et al. Distal congenital esophageal stenosis associated with esophageal atresia. J Pediatr Surg 1991; 26:478 - 82. [10] Ohkawa H, Takahashi H, Hoshino Y, et al. Lower esophageal stenosis in association with tracheobronchial remnants. J Pediatr Surg 1975;10: 453 - 7. [11] Shorter A, Mooney P, Vaccaro T, et al. Hydrostatic balloon dilation of congenital esophageal stenoses associated with esophageal atresia. J Pediatr Surg 2000;35:1742 - 5. [12] Roy G, Cohen R, Williams S. Endoscopic laser division of an esophageal web in a child. J Pediatr Surg 1996;31:439 - 40. [13] Kawahara H, Takaharu O, Okuyama H, et al. Esophageal motor function in congenital esophageal stenosis. J Pediatr Surg 2003;38: 1716 - 9.
www.elsevier.com/locate/jpedsurg
Thoracoscopic approach for congenital esophageal stenosis Marcelo Martinez-Ferro*, Martin Rubio, Lisandro Piaggio, Pablo Laje Department of Pediatric Surgery, J.P. Garrahan National Children’s Hospital, Buenos Aires, Argentina Index words: Congenital esophageal stenosis; Esophageal dilation; Thoracoscopy
Abstract Congenital esophageal stenosis (CES) is an infrequent entity; however, many cases have been reported during the last years. Its incidence falls between 1 per 25,000 and 1 per 50,000 live births and is associated with other congenital malformations in 17% to 33% of cases (mainly esophageal atresia). Congenital esophageal stenosis is defined as an intrinsic alteration of the esophageal wall given by the presence of ectopic tracheobronchial tissue, membranous diaphragm, muscular hypertrophy, or diffuse fibrosis of the submucosa, among other causes. The therapeutic options include endoscopic dilation and resection plus anastomosis (by either laparotomy or thoracotomy, depending on the level of the stenosis). We present the case of a 1-month-old baby boy with a CES located in the distal esophagus that is associated with anophthalmia and micropenis. We treated the lesion by means of a thoracoscopic resection of the affected segment and an esophageal end-to-end anastomosis. The patient’s long-term outcome was uneventful. As far as we know, this is the first report on thoracoscopic resolution of a CES. D 2006 Elsevier Inc. All rights reserved.
Although congenital esophageal stenosis (CES) is an infrequent entity, many cases have been reported during the last years. Its incidence falls between 1 per 25,000 and 1 per 50,000 live births and is associated with other congenital malformations in approximately 17% to 33% of cases (mainly esophageal atresia [EA]) [1,2]. The preoperative diagnosis is sometimes difficult, and the surgical approach is always controversial [3]. We report on the case of a CES successfully treated by means of a thoracoscopic approach.
1. Case report A 1-month-old baby boy was admitted at our institution because of severe aspirative pneumonia. He was an ex 36-weeker diagnosed with bilateral anophthalmia and micro* Corresponding author. Tel.: +54 11 4790 5709; fax: +54 11 4702 3116. E-mail address: [email protected] (M. Martinez-Ferro). 0022-3468/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2006.06.022
penis. He had had respiratory symptoms during and after feedings and had been fed through a nasogastric tube for the last weeks before his admission. Plain abdominal x-rays showed a big gastric chamber. In an upper gastrointestinal contrast study, a severe stenosis of the distal esophagus was found, 3 cm in length, with a normal esophageal anatomy both proximal and distal to the lesion (Fig. 1).
1.1. Surgical technique After an unsuccessful attempt to dilate the stenosis with an 8-mm pneumatic balloon, we decided on surgical correction. The patient was placed in the left lateroventral decubitus position. We used 3 ports: a 4-mm port was placed in the 5th intercostal (IC) space/midaxillary line for the 4-mm 308 scope (Karl Storz 28731 BWA, Tuttlingen, Germany); a 5-mm port was placed high in the armpit area; and a 3-mm port was placed in the 8th IC space/posterior axillary line. A 5-mm Hg CO2 pneumothorax was established. Short (20 cm) 3-mm instruments were used
E6
M. Martinez-Ferro et al.
Fig. 3 Microscopic picture (hematoxylin and eosin) of the resected stenotic esophagus. Significant muscular hypertrophy and submucosal fibrosis of the esophageal wall are observed. Fig. 1 Initial barium swallow study. A long stenotic segment is observed in the distal esophagus.
to dissect the esophagus free. No clear evidence of the stricture location could be externally observed. At that moment, an endoesophageal balloon (3-Fr Fogarty balloon catheter) was introduced through the mouth by the anesthesiologist. The balloon was inflated distally and proximally until the stenotic area was clearly identified. We then concluded the esophagus dissection by sparing both vagus nerves and resecting the stricture (Fig. 2). An end-toend esophageal anastomosis was carried out using 5/0 PDS extracorporeal knots. A nasogastric tube was placed before finishing the anastomosis to feed the baby soon after the
surgery. A 12-Fr chest tube was placed through the inferior port site.
1.2. Follow-up Six days after the procedure, an esophagogram showed no leak and good progression of the contrast through the anastomotic site. We pulled the chest tube out and started feeding the baby. The pathology report was consistent with muscular hypertrophy and submucosal fibrosis of the esophageal wall (Fig. 3). One month after surgery, we dilated a slight stenosis of the anastomotic site with a 12mm-long pneumatic balloon. Two months after surgery, the contrast study findings were normal. Four months after surgery, although the anatomy and clearance were normal, severe gastroesophageal reflux (GER) was diagnosed by means of a barium contrast study. The 24-hour pH-probe study findings were abnormal as well. Antireflux measures and medication were started, but a laparoscopic Nissen fundoplication was performed on the baby when he was 6 months old. The patient’s recovery was uneventful, and he was discharged 6 days after surgery. The long-term followup is normal in terms of growth and nutrition.
2. Discussion
Fig. 2 Thoracoscopic view of the dissected esophagus. A balloon (B) has been inflated inside the esophageal lumen. Cephalic traction of the balloon delimitates the inferior aspect of the stenosis (Sten). Diaph indicates diaphragm; Vert, vertebrae.
Congenital esophageal stenosis is defined as the presence of an intrinsic esophageal stenosis caused by an abnormality in the structure of the esophageal wall [4,5]. The stenosis may or may not be symptomatic from the first days after birth. Most cases have been described in the lower esophagus [6], but some authors have reported higher lesions and multiple lesions in a single esophagus [7]. The stenosis may be caused by ectopic tracheobronchial tissue, membranous webs, hypertrophy of the muscular layer, or fibrosis of the submucosa [8].
Thoracoscopic approach for congenital esophageal stenosis Because most cases of pediatric esophageal stenosis are secondary to the repair of EAs or a complication of GER, these and other causes of secondary esophageal stenosis must be ruled out (eg, extrinsic compressions, foreign bodies) [9]. Those cases of CES associated with EA usually have tracheobronchial remnants in the esophageal wall [10]. Once the diagnosis of CES is confirmed, other abnormalities that may be related to it must also be searched for (eg, cardiac and intestinal malformations, chromosomal anomalies). We first attempted to treat our patient following the guidelines of Shorter et al [11] (ie, by means of dilations). However, because tracheobronchial ectopic remnants tend to be difficult to stretch, we failed and so decided to treat the patient surgically. Some authors have reported successful dilations in up to 42.9% of cases, but the results may vary depending on the histologic findings. In the case of esophageal webs, endoscopic and open perforation-dilation procedures have been described [12]. Kawahara et al [13] reported a high incidence of GER in patients with CES, probably caused by loss of the natural high-pressure zone after surgery. This was the case in our patient. As far as we know, this is the first report on thoracoscopic resolution of a CES. We think that it is a safe, effective, and reproducible technique.
References [1] Olguner M, Ozdemir T, Akgur F, et al. Congenital esophageal stenosis owing to tracheobronchial remnants: a case report. J Pediatr Surg 1997;32:1485 - 7.
E7 [2] Vasudevan S, Kerendi F, Lee H, et al. Management of congenital esophageal stenosis. J Pediatr Surg 2002;37:1024 - 6. [3] Takamizawa S, Tsugawa C, Mouri N, et al. Congenital esophageal stenosis: therapeutic strategy based on etiology. J Pediatr Surg 2002; 37:197 - 201. [4] Amae S, Nio M, Kamiyama T, et al. Clinical characteristics and management of congenital esophageal stenosis: a report on 14 cases. J Pediatr Surg 2003;38:565 - 70. [5] Ramesh J, Ramanujam T, Jayaram G. Congenital esophageal stenosis: report of three cases, literature review, and a proposed classification. Pediatr Surg Int 2001;17:188 - 92. [6] Karaguzel G, Tanyel C, Akcoren Z, et al. Intramural tracheobronchial remnants associated with esophageal atresia: diagnostic aids. Pediatr Surg Int 1993;8:138 - 9. [7] Grabowski T, Andrews DA. Upper esophageal stenosis: two case reports. J Pediatr Surg 1996;31:1438 - 9. [8] Murphy G, Yazbeck S, Russo P. Isolated congenital esophageal stenosis. J Pediatr Surg 1995;30:1238 - 41. [9] Neilson R, Croitoru P, Guttman F, et al. Distal congenital esophageal stenosis associated with esophageal atresia. J Pediatr Surg 1991; 26:478 - 82. [10] Ohkawa H, Takahashi H, Hoshino Y, et al. Lower esophageal stenosis in association with tracheobronchial remnants. J Pediatr Surg 1975;10: 453 - 7. [11] Shorter A, Mooney P, Vaccaro T, et al. Hydrostatic balloon dilation of congenital esophageal stenoses associated with esophageal atresia. J Pediatr Surg 2000;35:1742 - 5. [12] Roy G, Cohen R, Williams S. Endoscopic laser division of an esophageal web in a child. J Pediatr Surg 1996;31:439 - 40. [13] Kawahara H, Takaharu O, Okuyama H, et al. Esophageal motor function in congenital esophageal stenosis. J Pediatr Surg 2003;38: 1716 - 9.