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Extracellular matrix for repair of type IV laryngotracheo-esophageal cleft
International Journal of Pediatric Otorhinolaryngology 79 (2015) 2484–2486
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case Report
Extracellular matrix for repair of type IV laryngotracheo-esophageal cleft Adele K. Evans a,*, Neal D. Kon b,1 a
Pediatric Otolaryngology – Head & Neck Surgery, Wake Forest1 School of Medicine, WFUBMC-OHNS, Medical Center Blvd., Winston-Salem, NC 27157, United States b Cardiothoracic Surgery, Wake Forest1 School of Medicine, WFUBMC-CT, Medical Center Blvd., Winston-Salem, NC 27157, United States
A R T I C L E I N F O
A B S T R A C T
Article history: Received 8 September 2015 Received in revised form 30 October 2015 Accepted 31 October 2015 Available online 6 November 2015
Type IV laryngotracheo-esophageal cleft (LTEC) extending to the level of the carina presents unique challenges to operative repair, particularly with respect to soft tissue durability. This is the first report of CorMatrix1 extra-cellular matrix (ECM) material use as an interposition graft in a four-layered LTEC repair. At day seven post-operatively, there was epithelialization along the surface of the trachea. At 3 months, she was stable for tracheotomy. At 6 months, the posterior wall resembled completely native tissue. CorMatrix1 ECM1 use intra-operatively and post-operative outcome were both highly satisfactory. No adverse reaction was seen in this case through 12-month follow up. ß 2015 Elsevier Ireland Ltd. All rights reserved.
Keywords: Laryngotracheo-esophageal cleft Extra-cellular matrix Airway reconstruction Tracheo-esophageal septum
1. Introduction Laryngotracheoesophageal cleft (LTEC) is a rare congenital disorder resulting from arrested development of the tracheoesophageal septum and failed posterior fusion of the cricoid lamina during embryogenesis, generating a spectrum of disease. The most commonly referenced classification scheme was proposed by Benjamin and Inglis [1]. Type IV LTEC extends distally into the thorax, to or beyond the carina. Presentation is primarily through respiratory symptoms, including aspiration, recurrent pneumonia, stridor, and cyanosis [2]. Type IV LTEC is associated with significant morbidity and mortality [3]. Repair presents unique challenges to the anesthetic, surgical and critical care teams. Stability of the esophageal and tracheal suture lines is a particular concern. Historically surgical repairs involve two-layered closure and three-layered closure with various interpositional grafts [2,4,5]. CorMatrix1 ECM1 (CorMatrix1 Cardiovascular, Inc. Roswell, GA) is a de-cellularized extracellular material derived from porcine small intestine. It is FDA-approved for the replacement of
* Corresponding author. Tel.: +1 336 716 3648; fax: +1 336 716 3857. E-mail addresses: [email protected] (A.K. Evans), [email protected] (N.D. Kon). 1 Tel.: +1 336 716 2124; fax: +1 336 716 3348. http://dx.doi.org/10.1016/j.ijporl.2015.10.058 0165-5876/ß 2015 Elsevier Ireland Ltd. All rights reserved.
pericardial, cardiac, and carotid vascular tissues. We describe the use of CorMatrix1 ECM1 as an interposition graft in a fourlayered repair of a Type IV LTEC. 2. Case report A full-term newborn female was transferred to the Neonatal Intensive Care Unit on the first day of life for evaluation for suspected esophageal atresia (EA) and tracheo-esophageal fistula (TEF). A replogle tube passed easily into the stomach and was visualized in the left upper quadrant; air insufflation resulted in distal bowel gas pattern on radiograph, decreasing suspicion of EA. On day of life 5, barium esophagram with 5 mL of barium contrast introduced through the replogle resulted in immediate reflux with aspiration, resulting in acute respiratory failure and intubation (Fig. 1). Presence of an H-type TEF was then assumed and repair was planned for the following day. During the case, a large air leak was noted from the 3.0 cuffless endotracheal tube (ETT). Diagnostic direct laryngoscopy and rigid bronchoscopy using a 4 mm 08 Hopkins telescope and the Storz AIDA1 system revealed LTEC extending to the carina (Fig. 2); the mainstem bronchi appeared widely patent but no membranous trachea was present. The planned surgery was aborted. On day of life 12, she underwent Nissen fundoplication and gastro-jejunostomy tube placement, then trans-tracheal primary surgical repair with a three-layered closure using clavicular perichondrium. She
A.K. Evans, N.D. Kon / International Journal of Pediatric Otorhinolaryngology 79 (2015) 2484–2486
Fig. 1. Radiographic image of trachea-bronchial tree contrasted by barium during barium esophagram via replogle instillation of 5 mL of barium contrast.
was intubated with a 3.0 cuffed naso-ETT without inflation of the cuff, but presence for emergent resuscitation if needed. Direct laryngoscopy and bronchoscopy on post-operative day 7 revealed complete dehiscence of the repair in the setting of foregut atony, severe bilious gastroesophageal reflux, and cuff inflation. Interval repair was planned in 6 weeks but with concerns for soft tissue quantity and integrity compromise by the wound dehiscence. CorMatrix1 ECM1 (CorMatrix1 Cardiovascular, Inc. Roswell, GA) is a de-cellularized extracellular material derived from porcine small intestine FDA-approved for the replacement of pericardial, cardiac, and carotid vascular tissues. In the case of this off-label surgical use, CorMatrix1 was used to reinforce the extraluminal side of the esophageal and posterior tracheal wall suture lines, creating four layers. With the patient on ECMO, a cricotracheal separation achieved complete exposure beyond the distal extent of the defects. The esophagus was closed primarily with a running continuous stitch with the mucosal edges imbricated using the 8-0 dyed Vicryl; the suture line was covered extraluminally with saline-moistened CorMatrix1 trimmed to 3.5 cm length at 6 mm width tacked into position inferiorly, laterally and superiorly with 7-0 violet PDS. The trachea was closed with 7.0 dyed Vicryl in a running suture, reinforced extraluminally with the CorMatrix1, trimmed to 3.5 cm 6 mm and tacked into position with 7-0 PDS with an inferiorly-based running stitch along the perimeter of the CorMatrix1 tied with a square knot at the superior margin (Fig. 3). She was intubated with a 3.5 uncuffed ETT for emergency access and pulmonary hygiene; she was unventilated on ECMO upon transfer to the PICU. On post-operative day 5, progressive ECMO-associated coagulopathy warranted bronchoscopy with clot removal to assess the
Fig. 2. Appearance of the cleft on tracheobronchoscopy; posteriorly the Dobhoff feeding tube is seen running through the esophagus and anteriorly the bronchi are visible at the base of the defect right at the carina.
2485
Fig. 3. Intraoperative appearance of the transected trachea attached to the Prolene suture at the 12 o’clock position and the esophageal extraluminal layer of CorMatrix1 tacked into position with 7-0 PDS posteriorly.
wound-healing for potential early ECMO-decannulation. The tracheo-esophageal repair was adequately healed to commence lung-recruitment in preparation for decannulation. Jet-ventilation was instituted and pulmonary recruitment was complete to tolerate decannulation from ECMO on day 10. The patient was on conventional ventilation with FiO2 40%, PEEP 5, clear breath sounds and good bilateral aeration on chest radiograph with sedation weaned for successful extubation on post-operative day 20 in the operating room during laryngoscopy, bronchoscopy and esophagoscopy. Her course was later complicated by dependence upon high doses of sedating medication, severe left mainstem bronchomalacia and tracheomalacia. Tracheotomy was ultimately required when she had adequate tracheal length; a customized cuffless Bivona Flex-Tend tube was used. She was discharged to home care 1 month after tracheotomy with continuous home ventilation at 6 months of age. Bronchoscopy performed at week 3 (Video 1) demonstrated an asymptomatic minor mucosal dehiscence in the inferior trachea that spontaneously resolved with mucosalization over the CorMatrix1 ECM1 by week 6 with ciliated epithelium present on TEM and histopathologic specimen of tracheal biopsies. Bronchoscopy at month 3 (Fig. 4), month 6 (Fig. 5) and month 12 revealed resolution of ETT-associated inflammation. Clinically the ECM1 was completely incorporated (Video 2). 3. Discussion Competing considerations for ventilation and oxygenation, nutrition, GERD, and soft tissue management make LTEC repair
Fig. 4. Three months post-operatively, the granulation from the intubation has resolved, there is a tiny posterior blind pouch but the posterior wall appears intact, albeit thinly mucosalized over the CorMatrix1.
2486
A.K. Evans, N.D. Kon / International Journal of Pediatric Otorhinolaryngology 79 (2015) 2484–2486
4. Conclusion CorMatrix1 ECM1 intra-operatively handling and use and its post-operative outcome were both highly satisfactory. No adverse reaction was seen in this case through 12 months of follow up. Disclosures This is an unfunded project. There are no financial disclosures to make and there are no conflicts of interest to disclose. All work was done at Brenner Children’s Hospital at Wake Forest1 Baptist Health. Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.ijporl.2015.10. 058. Fig. 5. Six months postoperatively, in-office flexible bronchoscopy through the tracheotomy reveals that the blind pouch has resolved and the posterior tracheal mucosa appears as thick as native tissue.
References
exceedingly complex. We used de-cellularized porcine ECM (CorMatrix1) as an interposition graft to reinforce suture lines extraluminally in a four-layered repair. This prevented exposure of either lumen to the mediastinum or to the opposing suture line in the event of mucosal dehiscence. It was durable. Being decellularized by osmosis, it was not preserved with toxic substances that could compromise cellular invasion and replacement. It ultimately took on an appearance of native tissue with excellent mucosalization on both luminal surfaces. We hypothesize that its positioning between well-vascularized tracheal and esophageal tissues containing muscle fibers supported its marked incorporation into the tissue without evidence of fibrosis or inflammation.
[1] B. Benjamin, A. Inglis, Minor congenital laryngeal clefts: diagnosis and classification, Ann. Otol. Rhinol. Laryngol. 98 (1989) 417–420. [2] R. Rahbar, I. Bouillon, G. Roger, A. Lin, R.C. Muss, F. Danyelle, et al., The presentation and management of laryngeal cleft: a 10-year experience, Arch. Otolaryngol. Neck Surg. 132 (2006) 1335–1341, http://dx.doi.org/10.1001/archotol.132.12.1335. [3] N.N. Mathura, G.J. Peek, C.M. Bailey, M.J. Elliott, Strategies for managing type IV laryngotracheo-esophageal clefts at Great Ormond Street Hospital for Children, Int. J. Pediatr. Otorhinolaryngol. 70 (2006) 1901–1910, http://dx.doi.org/10.1016/ j.ijporl.2006.06.017. [4] K. Geller, Y. Kim, J. Koempel, K.D. Anderson, Surgical management of type III and IV laryngotracheo-esophageal clefts: the three-layered approach, Int. J. Pediatr. Otorhinolaryngol. 74 (June (6)) (2010) 652–657, http://dx.doi.org/10.1016/j.ijporl. 2010.03.013. [5] E.J. Props, J.B. Ida, M.J. Rutter, Repair of long type IV posterior laryngeal cleft through a cervical approach using cricotracheal separation, Laryngoscope 123 (3) (2013) 801–804.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case Report
Extracellular matrix for repair of type IV laryngotracheo-esophageal cleft Adele K. Evans a,*, Neal D. Kon b,1 a
Pediatric Otolaryngology – Head & Neck Surgery, Wake Forest1 School of Medicine, WFUBMC-OHNS, Medical Center Blvd., Winston-Salem, NC 27157, United States b Cardiothoracic Surgery, Wake Forest1 School of Medicine, WFUBMC-CT, Medical Center Blvd., Winston-Salem, NC 27157, United States
A R T I C L E I N F O
A B S T R A C T
Article history: Received 8 September 2015 Received in revised form 30 October 2015 Accepted 31 October 2015 Available online 6 November 2015
Type IV laryngotracheo-esophageal cleft (LTEC) extending to the level of the carina presents unique challenges to operative repair, particularly with respect to soft tissue durability. This is the first report of CorMatrix1 extra-cellular matrix (ECM) material use as an interposition graft in a four-layered LTEC repair. At day seven post-operatively, there was epithelialization along the surface of the trachea. At 3 months, she was stable for tracheotomy. At 6 months, the posterior wall resembled completely native tissue. CorMatrix1 ECM1 use intra-operatively and post-operative outcome were both highly satisfactory. No adverse reaction was seen in this case through 12-month follow up. ß 2015 Elsevier Ireland Ltd. All rights reserved.
Keywords: Laryngotracheo-esophageal cleft Extra-cellular matrix Airway reconstruction Tracheo-esophageal septum
1. Introduction Laryngotracheoesophageal cleft (LTEC) is a rare congenital disorder resulting from arrested development of the tracheoesophageal septum and failed posterior fusion of the cricoid lamina during embryogenesis, generating a spectrum of disease. The most commonly referenced classification scheme was proposed by Benjamin and Inglis [1]. Type IV LTEC extends distally into the thorax, to or beyond the carina. Presentation is primarily through respiratory symptoms, including aspiration, recurrent pneumonia, stridor, and cyanosis [2]. Type IV LTEC is associated with significant morbidity and mortality [3]. Repair presents unique challenges to the anesthetic, surgical and critical care teams. Stability of the esophageal and tracheal suture lines is a particular concern. Historically surgical repairs involve two-layered closure and three-layered closure with various interpositional grafts [2,4,5]. CorMatrix1 ECM1 (CorMatrix1 Cardiovascular, Inc. Roswell, GA) is a de-cellularized extracellular material derived from porcine small intestine. It is FDA-approved for the replacement of
* Corresponding author. Tel.: +1 336 716 3648; fax: +1 336 716 3857. E-mail addresses: [email protected] (A.K. Evans), [email protected] (N.D. Kon). 1 Tel.: +1 336 716 2124; fax: +1 336 716 3348. http://dx.doi.org/10.1016/j.ijporl.2015.10.058 0165-5876/ß 2015 Elsevier Ireland Ltd. All rights reserved.
pericardial, cardiac, and carotid vascular tissues. We describe the use of CorMatrix1 ECM1 as an interposition graft in a fourlayered repair of a Type IV LTEC. 2. Case report A full-term newborn female was transferred to the Neonatal Intensive Care Unit on the first day of life for evaluation for suspected esophageal atresia (EA) and tracheo-esophageal fistula (TEF). A replogle tube passed easily into the stomach and was visualized in the left upper quadrant; air insufflation resulted in distal bowel gas pattern on radiograph, decreasing suspicion of EA. On day of life 5, barium esophagram with 5 mL of barium contrast introduced through the replogle resulted in immediate reflux with aspiration, resulting in acute respiratory failure and intubation (Fig. 1). Presence of an H-type TEF was then assumed and repair was planned for the following day. During the case, a large air leak was noted from the 3.0 cuffless endotracheal tube (ETT). Diagnostic direct laryngoscopy and rigid bronchoscopy using a 4 mm 08 Hopkins telescope and the Storz AIDA1 system revealed LTEC extending to the carina (Fig. 2); the mainstem bronchi appeared widely patent but no membranous trachea was present. The planned surgery was aborted. On day of life 12, she underwent Nissen fundoplication and gastro-jejunostomy tube placement, then trans-tracheal primary surgical repair with a three-layered closure using clavicular perichondrium. She
A.K. Evans, N.D. Kon / International Journal of Pediatric Otorhinolaryngology 79 (2015) 2484–2486
Fig. 1. Radiographic image of trachea-bronchial tree contrasted by barium during barium esophagram via replogle instillation of 5 mL of barium contrast.
was intubated with a 3.0 cuffed naso-ETT without inflation of the cuff, but presence for emergent resuscitation if needed. Direct laryngoscopy and bronchoscopy on post-operative day 7 revealed complete dehiscence of the repair in the setting of foregut atony, severe bilious gastroesophageal reflux, and cuff inflation. Interval repair was planned in 6 weeks but with concerns for soft tissue quantity and integrity compromise by the wound dehiscence. CorMatrix1 ECM1 (CorMatrix1 Cardiovascular, Inc. Roswell, GA) is a de-cellularized extracellular material derived from porcine small intestine FDA-approved for the replacement of pericardial, cardiac, and carotid vascular tissues. In the case of this off-label surgical use, CorMatrix1 was used to reinforce the extraluminal side of the esophageal and posterior tracheal wall suture lines, creating four layers. With the patient on ECMO, a cricotracheal separation achieved complete exposure beyond the distal extent of the defects. The esophagus was closed primarily with a running continuous stitch with the mucosal edges imbricated using the 8-0 dyed Vicryl; the suture line was covered extraluminally with saline-moistened CorMatrix1 trimmed to 3.5 cm length at 6 mm width tacked into position inferiorly, laterally and superiorly with 7-0 violet PDS. The trachea was closed with 7.0 dyed Vicryl in a running suture, reinforced extraluminally with the CorMatrix1, trimmed to 3.5 cm 6 mm and tacked into position with 7-0 PDS with an inferiorly-based running stitch along the perimeter of the CorMatrix1 tied with a square knot at the superior margin (Fig. 3). She was intubated with a 3.5 uncuffed ETT for emergency access and pulmonary hygiene; she was unventilated on ECMO upon transfer to the PICU. On post-operative day 5, progressive ECMO-associated coagulopathy warranted bronchoscopy with clot removal to assess the
Fig. 2. Appearance of the cleft on tracheobronchoscopy; posteriorly the Dobhoff feeding tube is seen running through the esophagus and anteriorly the bronchi are visible at the base of the defect right at the carina.
2485
Fig. 3. Intraoperative appearance of the transected trachea attached to the Prolene suture at the 12 o’clock position and the esophageal extraluminal layer of CorMatrix1 tacked into position with 7-0 PDS posteriorly.
wound-healing for potential early ECMO-decannulation. The tracheo-esophageal repair was adequately healed to commence lung-recruitment in preparation for decannulation. Jet-ventilation was instituted and pulmonary recruitment was complete to tolerate decannulation from ECMO on day 10. The patient was on conventional ventilation with FiO2 40%, PEEP 5, clear breath sounds and good bilateral aeration on chest radiograph with sedation weaned for successful extubation on post-operative day 20 in the operating room during laryngoscopy, bronchoscopy and esophagoscopy. Her course was later complicated by dependence upon high doses of sedating medication, severe left mainstem bronchomalacia and tracheomalacia. Tracheotomy was ultimately required when she had adequate tracheal length; a customized cuffless Bivona Flex-Tend tube was used. She was discharged to home care 1 month after tracheotomy with continuous home ventilation at 6 months of age. Bronchoscopy performed at week 3 (Video 1) demonstrated an asymptomatic minor mucosal dehiscence in the inferior trachea that spontaneously resolved with mucosalization over the CorMatrix1 ECM1 by week 6 with ciliated epithelium present on TEM and histopathologic specimen of tracheal biopsies. Bronchoscopy at month 3 (Fig. 4), month 6 (Fig. 5) and month 12 revealed resolution of ETT-associated inflammation. Clinically the ECM1 was completely incorporated (Video 2). 3. Discussion Competing considerations for ventilation and oxygenation, nutrition, GERD, and soft tissue management make LTEC repair
Fig. 4. Three months post-operatively, the granulation from the intubation has resolved, there is a tiny posterior blind pouch but the posterior wall appears intact, albeit thinly mucosalized over the CorMatrix1.
2486
A.K. Evans, N.D. Kon / International Journal of Pediatric Otorhinolaryngology 79 (2015) 2484–2486
4. Conclusion CorMatrix1 ECM1 intra-operatively handling and use and its post-operative outcome were both highly satisfactory. No adverse reaction was seen in this case through 12 months of follow up. Disclosures This is an unfunded project. There are no financial disclosures to make and there are no conflicts of interest to disclose. All work was done at Brenner Children’s Hospital at Wake Forest1 Baptist Health. Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.ijporl.2015.10. 058. Fig. 5. Six months postoperatively, in-office flexible bronchoscopy through the tracheotomy reveals that the blind pouch has resolved and the posterior tracheal mucosa appears as thick as native tissue.
References
exceedingly complex. We used de-cellularized porcine ECM (CorMatrix1) as an interposition graft to reinforce suture lines extraluminally in a four-layered repair. This prevented exposure of either lumen to the mediastinum or to the opposing suture line in the event of mucosal dehiscence. It was durable. Being decellularized by osmosis, it was not preserved with toxic substances that could compromise cellular invasion and replacement. It ultimately took on an appearance of native tissue with excellent mucosalization on both luminal surfaces. We hypothesize that its positioning between well-vascularized tracheal and esophageal tissues containing muscle fibers supported its marked incorporation into the tissue without evidence of fibrosis or inflammation.
[1] B. Benjamin, A. Inglis, Minor congenital laryngeal clefts: diagnosis and classification, Ann. Otol. Rhinol. Laryngol. 98 (1989) 417–420. [2] R. Rahbar, I. Bouillon, G. Roger, A. Lin, R.C. Muss, F. Danyelle, et al., The presentation and management of laryngeal cleft: a 10-year experience, Arch. Otolaryngol. Neck Surg. 132 (2006) 1335–1341, http://dx.doi.org/10.1001/archotol.132.12.1335. [3] N.N. Mathura, G.J. Peek, C.M. Bailey, M.J. Elliott, Strategies for managing type IV laryngotracheo-esophageal clefts at Great Ormond Street Hospital for Children, Int. J. Pediatr. Otorhinolaryngol. 70 (2006) 1901–1910, http://dx.doi.org/10.1016/ j.ijporl.2006.06.017. [4] K. Geller, Y. Kim, J. Koempel, K.D. Anderson, Surgical management of type III and IV laryngotracheo-esophageal clefts: the three-layered approach, Int. J. Pediatr. Otorhinolaryngol. 74 (June (6)) (2010) 652–657, http://dx.doi.org/10.1016/j.ijporl. 2010.03.013. [5] E.J. Props, J.B. Ida, M.J. Rutter, Repair of long type IV posterior laryngeal cleft through a cervical approach using cricotracheal separation, Laryngoscope 123 (3) (2013) 801–804.