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The Laryngoscope C 2013 The American Laryngological, V
Rhinological and Otological Society, Inc.
Case Report
Repair of Long Type IV Posterior Laryngeal Cleft Through a Cervical Approach Using Cricotracheal Separation Evan J. Propst, MD; Jonathan B. Ida, MD; Michael J. Rutter, MD
A female infant with CHARGE syndrome and a long type IV cleft extending to within 5 mm of the carina underwent transcervical repair at 5 weeks of age. The trachea was transected from the cricoid cartilage and was peeled off the esophagus. The front of the esophagus and the back of the trachea were repaired while still ventilating the patient. The trachea was reconnected to the cricoid cartilage. This technique obviated the need for a sternal split, thoracotomy, cardiopulmonary bypass, or extracorporeal membrane oxygenation. It improved visibility, access, airway stability, and coverage of the anastomosis with periosteum permitting a three-layer closure. Key Words: Laryngeal cleft, LTE cleft, long type IV, type 4, cervical approach, cricotracheal separation, tibial periosteum, sternal periosteum, aspiration. Laryngoscope, 000:000–000, 2013 3
INTRODUCTION Laryngeal clefts are rare congenital anomalies resulting from failure of fusion of the laryngotracheal groove during embryogenesis. These anomalies differ anatomically with respect to the distal extent of the cleft and are generally classified according to the well-recognized system proposed by Benjamin and Inglis.1 These authors describe four cleft subtypes, with type IV referring to a cleft extending to the thoracic trachea. Based on the experience of the senior author (M.J.R.), a modification was made to this classification system that allowed for differing severities of type IV clefts.2,3 This revised classification distinguishes between long and short type IV clefts because the management and prognosis for type IV clefts extending close to the carina are markedly different from those of other type IV clefts.
From the Department of Otolaryngology–Head and Neck Surgery (E.J.P.), University of Toronto, Toronto, Ontario, Canada and Department of Otolaryngology–Head and Neck Surgery (E.J.P.), Hospital for Sick Children, Toronto, Ontario, Canada; the Division of Pediatric Otolaryngology–Head (J.B.I.), Lurie Children’s Hospital, Chicago, IL; the Division of Peditric Otolarygology–Head and Neck Surgery (M.J.R.), Cincinnati Children’s Hospital Medical Center, Cincinnati, OH; and the Department of Otolaryngology–Head and Neck Surgery ( M . J . R .), University of Cincinnati College of Medicine, Cincinnati, OH, U.S.A. Editor’s Note: This Manuscript was accepted for publication July 19, 2012. The authors have no funding, financial relationships, or conflicts of interest to disclose. Send correspondence to Michael J. Rutter, FRACS, Division of Pediatric Otolaryngology–Head and Neck Surgery, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229-3039. E-mail:
[email protected] DOI: 10.1002/lary.23660
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Infants with a long type IV cleft are particularly challenging to manage.4–6 Severe aspiration is inevitable. Airway maintenance may be problematic as the endotracheal tube may reside in the esophagus or trachea, with the ‘‘tracheoesophagus’’ being a common cavity. In addition, these children usually have associated microgastria and severe gastroesophageal reflux disease, as well as other airway and non-airway congenital anomalies. Currently, there is no consensus as to the best management strategy for these complex patients, with dilemmas relating both to issues of access and oxygenation. Access may be through a lateral thoracotomy approach, a transsternal approach, or a cervical approach, either transtracheally or from an extended lateral pharyngotomy. Oxygenation may be achieved via intubation, extracorporeal membrane oxygenation, or cardiopulmonary bypass. Having had experience with all of these dilemmas (2 via transsternal transtracheal approach–1 on bypass and 1 on ECMO; 1 transcervical with partial sternotomy and lateral pharyngotomy; 1 transcervical transtracheal), our preference after operating on the patient described herein is to perform a transcervical repair and tracheal transection, permitting access to the cleft while maintaining oxygenation. We report our experience with this approach.
CASE REPORT A female infant born at 31 weeks of gestation was diagnosed with CHARGE syndrome and a long type IV cleft extending to within 5 mm of the carina (there were no concomitant cardiac malformations requiring surgery). The infant was initially stabilized with a 4.0 endotracheal tube (ETT) placed 5-mm proximal to the
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Fig. 1. A) View of long type IV posterior laryngeal cleft from glottic level on microlaryngoscopy. B) Closer view of trachea (solid white arrow), esophagus (dashed white arrow), and esophageal pouch (solid black arrow). C) Distal aspect of cleft (black arrow) terminating 5 mm proximal to carina.
apex of the cleft, and a repogle tube placed in the esophagus for suctioning. She nevertheless continued to experience multiple hypoxic events attributed to aspiration and required repeated airway clearance of thick mucus plugs with flexible bronchoscopy. At 4 weeks of age, she was transferred to our institution for definitive airway management. At initial evaluation, we confirmed the extent of the laryngeal cleft and also noted a posterior esophageal pouch at the level of the carina (Fig. 1). At 5 weeks of age, the infant underwent transcervical repair of the cleft (Fig. 2). The airway was initially secured and stabilized with a 3.0 ETT placed in the right mainstem bronchus. After identification of the recurrent laryngeal nerves, the upper trachea was exposed and transected at the lower border of the cricoid cartilage. The trachea distal to the transection was then ‘‘peeled up’’ off the
esophagus (Fig. 3). This was achieved by dividing the mucosa on either side of the cleft a few millimeters posterior to the apex of the tracheoesophageal mucosal junction. The dissection was continued down to the apex of the cleft and was extended a few millimeters into the space between the trachea and esophagus distal to the cleft. During this procedure, oxygenation was maintained by an endotracheal tube placed in the trachea with the tip of the tube lying in the right mainstem bronchus. Despite attempts to partially ventilate the left lung through the Murphy eye of the endotracheal tube that resided at the carinal level, the airway remained quite precarious. Therefore, the first step was to close the distal aspect of the trachea to provide a more stable enclosure in which the endotracheal tube tip could be placed. The proximal aspect of the laryngeal cleft was then dissected, commencing at the lower border of the
Fig. 2. Schematic of procedure from posterolateral view. A) Posterior laryngeal cleft extends from arytenoid cartilages to within 5 mm of the carina. B) Trachea is transected below cricoid and peeled anteriorly, separating it from the esophagus. The endotracheal tube is repositioned into the cut end of the trachea. C) Standing at the head of the bed, the distal anterior aspect of the esophagus is sutured closed. The trachealis muscle is sutured closed from a posterior approach while keeping the endotracheal tube in place. The superior aspect of the cleft is sutured, looking superiorly from below the cricoid. D) Tibial periosteum is placed over the sutured cleft between the trachea and the esophagus. E) Sternal periosteum is placed between the trachea and the esophagus to reinforce the cricotracheal anastomotic site. The patient is reintubated nasally.
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Fig. 3. A) View from the head of the bed looking distally down the posterior laryngeal cleft. The trachea (white arrow) has been separated from the cricoid cartilage and peeled anteriorly off the esophagus (black arrow). The endotracheal tube has been removed from the trachea for the photograph, and a different endotracheal tube has been placed in the esophagus to distend it to facilitate dissection (black arrow). B) The repaired distal trachea is reintubated (white arrow), and the remainder of the esophagus is to be repaired (black arrow).
cricoid cartilage and progressing proximally to the arytenoids. Next, the esophageal aspect of the cleft was repaired with interrupted sutures, with the knots placed in the esophageal lumen. At this point, tibial and sternal periosteal interposition grafts were placed from beyond the carina up to the arytenoids. The posterior trachea was then repaired in a distal to proximal fashion, with the repair performed from the posterior aspect of the trachea. This approach permitted oxygenation, as the endotracheal tube remained in position while the posterior trachea was repaired. The carina was not anchored superiorly because its anterior attachments were not disturbed using this technique. Once the distal trachea was repaired, the proximal tracheal mucosa was repaired from the lower border of the cricoid cartilage up to the arytenoids. The transected trachea was then reconnected to the cricoid cartilage. Bronchoscopy 2 months later confirmed the repair had healed well (Fig. 4) and the patient continues to be well clinically over 2 years later.
DISCUSSION The fundamental challenge in repairing long type IV clefts relates to both access and oxygenation. A trans-
cervical approach provides excellent access to most of the laryngeal cleft (albeit with limited space while working on the distal aspect of the repair). Because children with long type IV clefts typically have a short trachea, neck extension for access down to the carina is usually adequate. If improved access is desired, an upper sternal split through the manubrium may easily be performed using the transcervical approach. In most cases, the cleft proximal to the tracheal transection may also be repaired without performing a complete laryngofissure. In our experience, the limited space provided by this approach while working on the distal aspect of the cleft repair is comparable or better than the amount of space provided using lateral based approaches. A long interposition graft between the repaired trachea and esophagus is required, extending from the distal aspect of the cleft to the level of the cricoid cartilage. Although our patient had insufficient tibial periosteum to completely cover the entire length of the esophageal repair, necessitating placement of additional sternal periosteum, we now believe that an adequate amount of sternal periosteum alone would have been sufficient to span the entire distance required. Furthermore, sternal periosteum is easily harvested through a transcervical approach.
Fig. 4. Repaired posterior wall of the trachea after 2 months. A) Proximal trachea; B) Mid trachea; and C) Carina.
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A criticism of our approach (whereby the trachea is separated from the cricoid) is that repair of the tracheal mucosa necessitates a 4-point anastomosis at the lower border of the cricoid, placing it at a higher risk of dehiscence. However, a dehiscence in the cervical trachea would be less likely to cause mediastinitis and would therefore have less morbidity as compared with a dehiscence in the thoracic trachea. Furthermore, if the interposition graft and the esophageal mucosa are intact, a tracheal dehiscence would eventually heal. In the event that a tracheoesophageal fistula redevelops at this site, it is easier to manage and repair than a dehiscence close to the carina. Another concern might be that much of the blood supply to the trachea could theoretically be lost using this approach. However, after separation of the trachea from the cricoid and the esophagus in this patient, all mucosal edges in the tracheal lumen appeared to be bleeding and viable. Oxygenation and ventilation may be maintained with an ETT tube placed close to or past the carina, thereby obviating the need for extracorporeal membrane oxygenation or cardiopulmonary bypass. To minimize the amount of pressure placed on the suture line, we recommend managing the postoperative airway with a transnasal endotracheal tube. Although placement of a tracheotomy tube is inevitable (this patient was not ventilator dependent), delaying this for 2 or 3 weeks following surgery will decrease the chances that the tip of the tracheotomy tube will disrupt the integrity of the distal repair (the curve of a tracheotomy tube exerts more pressure on the posterior tracheal wall than a nasal endotracheal tube). A stormy postoperative course is routine in these children, especially due to the
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long segment of esophagus that ends up having uncoordinated motility. In addition, the tip of the endotracheal tube kept migrating into the right main bronchus (perhaps due to shortening of the trachea due to contraction during healing), necessitating bronchoscopy and repositioning of the endotracheal tube every few days.
CONCLUSION Long type IV posterior laryngeal clefts are extremely difficult to repair. Performing a tracheal separation with posterior repair via a cervical approach can obviate the need for a sternal split or a thoracotomy. This approach also improves visibility, access, and airway stability. Moreover, it may obviate the need for cardiopulmonary bypass or extracorporeal membrane oxygenation, and allows for better coverage of the anastomosis with periosteum, permitting a three-layer closure.
BIBLIOGRAPHY 1. Benjamin B, Inglis A. Minor congenital laryngeal clefts: diagnosis and classification. Ann Otol Rhinol Laryngol 1989;98:417–420. 2. Rutter MJ, Azizkhan RG, Cotton RT. Posterior laryngeal cleft. In: Ziegler MM, Azizkhan RG, Weber TR, eds. Operative Pediatric Surgery. New York, NY: McGraw-Hill, 2003. 3. Rutter MJ, Azizkhan RG. Posterior laryngeal cleft. In: Ziegler MM, Azizkhan RG, Weber TR, von Allmen D, eds. Operative Pediatric Surgery. 2nd ed. New York, NY: McGraw-Hill. In press. 4. Evans KL, Courteney-Harris R, Bailey CM, Evans JN, Parsons DS. Management of posterior laryngeal and laryngotracheoesophageal clefts. Arch Otolaryngol Head Neck Surg 1995;121:1380–1385. 5. Simpson BB, Ryan DP, Donahoe PK, et al. Type IV laryngotracheoesophageal clefts: surgical management for long-term survival. J Pediatr Surg 1996;31:1128–1133. 6. Mathur NN, Peek GJ, Bailey CM, Elliott MJ. Strategies for managing type IV laryngotracheoesophageal clefts at Great Ormond Street Hospital for Children. Int J Pediatr Otorhinolaryngol 2006;70:1901–1910.
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