Operative Strategy for Recurrent Laryngeal Cleft: A Case Report and Review of the Literature By D.K. Robie, R.H. Pearl, C. Gonsales,

R.D. Restuccia, and M.A. Hoffman

Washington, 0 A case is reported of recurrent laryngotracheoesophageal cleft following two attempted repairs. The anterior approach to the larynx and trachea provided excellent exposure for defining the extent of the recurrent cleft, and allowed precise anatomic repair. Neurovascular structures were easily avoided by this operative method. Of 170 clefts reported in the literature, 19 recurred necessitating reoperation. The anterior approach to the larynx and trachea is appropriate in recurrent clefts and may be preferable at the initial repair. Copyright o 1991 by W.B. Saunders Company INDEX WORDS:

Laryngotracheal

cleft, recurrent.

L

ARYNGEAL AND laryngotracheoesophageal (LTE) clefts have historically represented a diagnostic and therapeutic challenge to surgeons. Symptoms range from inspiratory stridor in short clefts to massive aspiration in long tracheoesophageal clefts. Diagnosis requires a high index of suspicion, accurate interpretation of barium esophograms, and thorough endoscopic evaluation. A standardized classification scheme is not yet available and accepted. We found 170 clefts reported in the literature, nineteen of which required reoperation.‘.” Accepted operative approaches include a posterior pharyngotomy, lateral pharyngotomy, anterior approach, and endoscopic repair depending on the extent of the cleft. Peterson19 performed the first successful repair of a type 1 cleft in 1955, whereas Donohoe and Gee6 described the first long-term survivor with a corrected type 3 cleft. We report a case of LTE cleft that recurred after two attempted repairs. Final closure was achieved through an anterior approach to the larynx and trachea. This approach avoided injury to neurovascular structures, and allowed precise repair of the cleft.

DC

kidney, 13 vertebrae, and fused left 10th and 11th ribs. Endoscopic evaluation showed residual upper laryngeal cleft with prominent esophageal bulging into the trachea. There were three recurrent TEFs each associated with a visible suture and distal tracheomalacia. At 9 months she underwent repair via an anterior approach. The trachea was split anteriorly exposing the laryngeal cleft and recurrent fistulas. The original defect was recreated by removing all sutures and splitting the posterior tracheal wall and anterior esophagus (Fig 1). The esophagus was closed with absorbable suture in two layers. The suture line was distracted from the midline (Fig 1). The trachea and entire laryngeal cleft were closed with interrupted absorbable suture as was the anterior trachea (Fig 2). Repeat endoscopy at 16 months postoperatively showed wellhealed suture lines with mobile vocal cords and no recurrent fistula. At present she continues to require continuous positive airway pressure support with occasional mechanical ventilation for residual distal tracheomalacia. No attempts at oral intake have been made. DISCUSSION

The original classification by PetersonY anatomically divided clefts into three types: type 1 involving the larynx alone, type 2 involving up to six tracheal rings, and type 3 extending to the carina. Armitage’ further subclassified type 1 clefts depending on involvement of the cricoid. Fuzesi and Young*’ proposed an embryologically based classification with four types of clefts: type 1 to the cricoid, type 2 involving the cricoid, type 3 into the cervical trachea, and type 4 into the thoracic trachea. Failure of development and fusion of the interarytenoid muscle and cricoid cartilage results in type 1 and 2 clefts. Failure of inward migration and fusion of the tracheoesophageal folds results in type 3 and 4 clefts. Evans3 recommends a clinical classification based on presenting symptoms: type 1 clefts confined to supraglottic and presenting with inspiratory stridor; type 2 clefts

CASE REPORT A 1,506-g girl was born in October 1988 at 33 weeks’ gestation. An H-type tracheoesophageal fistula (TEF) was found and repaired through a combined thoracic and lateral cervical approach on day one of life. Postoperatively, she required prolonged mechanical ventilation. Follow-up bronchoscopy demonstrated an LTF cleft, and at 2 months she underwent further repair via a lateral cervical approach. A Nissen fundoplication and jejunostomy were also performed. Postoperatively, continued ventilatory dependence prompted a tracheostomy. A barium esophagram at 6 months of age showed recurrent tracheoesophageal fistulas. The patient was transferred to Walter Reed Army Medical Center for further evaluation. An asymptomatic atrial septal defect and large right ventricle with dysplastic tricuspid valve were noted. There was a small right Journalof Pediatric Surgery, Vol26, No 8 (August), 1991: pp 971-974

From the Department of Surgery, Walter Reed Army Medical Center, Washington, DC. Presented at the Jens G. Rosenkrantz Resident Competition at the 42nd Annual Meeting of the Surgical Section of the American Academy of Pediatrics, Boston, Massachusetts October 6-7, 1990. The opinions and conclusionspresented are those of the authors and do not necessatily represent the views of the Army Medical Deparzment, the Depanment of the Army, or any other US Government agency. Address reprint requests to Richard H. Pearl, MD, Chief; Pediatric General Surgery Service, Walter Reed Army Medical Center, Washington, DC 20307. Copyright o 1991 by W.B. Saunders Company 0022-3468/9112608-0016$03.00/0

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posed disadvantages of this approach include poor access to long LTE clefts, laryngotracheal instability, and inhibitory effects on laryngeal growth and development. However, long type 3 clefts require right thoracotomy for complete repair regardless of the cervical approach. Laryngotracheal instability is predicted especially in the presence of failed posterior cricoid fusion. This is not noted in the literature with postoperative ventilatory dependence usually due to distal tracheomalacia distant from the cleft repair. Calcaterra et a125found no difference in laryngeal growth and function in dogs 8 months after anterior laryngofissure. Placement of an anterior keel for 6 weeks postoperatively interfered with thyroid apposition that was not clinically significant. Montgomery and Smith26 successfully repaired a cleft in an adult by this approach. Cotton et al* recommend this approach routinely in the initial repair of all clefts. We agree and recommend the anterior approach for repair of most recurrent clefts. Of 170 clefts reported, 19 required reoperation for recurrence. Many patients required multiple operations through different approaches to achieve closure; a total of 48 operations were performed with

Fig 1. Exposure was acheived by splitting the anterior wall of the larynx and trachea in the midline. The recurrent upper cleft was easily identified as were the three recurrent TEFs each containing a nonabsorbable suture.

involving the cricoid and cervical trachea presenting with coughing, choking, and aspiration pneumonia; and type 3 involving the thoracic trachea causing severe respiratory compromise. The most recent classification proposed by Cotton et al* divides clefts into laryngeal and LTE clefts depending on length. The three standard operative approaches are the anterior approach to the larynx and trachea, lateral pharyngotomy, and posterior pharyngotomy. The anterior approach was first described by Jahrsdoerfer et al” in 1967 for a cleft extending into the cervical trachea. Bell et al” repaired a 4-cm cleft to the fifth tracheal ring via an anterior approach. The advantages of this approach include excellent exposure, identification of the entire defect, precise anatomic closure, and avoidance of neurovascular structures. This latter advantage is particularly important for reoperative cleft surgery in which scarring and distortion of tissue planes is present. The superior and inferior laryngeal arteries are the primary blood supply to the larynx and trachea and course laterally in close approximation to the superior and recurrent laryngeal nerves. A nonrecurrent laryngeal nerve may further complicate the lateral approach.Z3.24The pro-

Fig 2. The original cleft was recreated by removing all sutures from the anterior esophagus and posterior trachea. The esophageal wall was closed with continuous absorbable suture. The posterior and anterior larynx and trachea were closed with interrupted absorbable suture. As shown in the inset, the esophgeal suture line was deviated from the midline with additional sutures.

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deaths. Evans3z4reported two type 1 clefts that remained open despite six reoperations and two type 2 clefts requiring five and two operations, respectively, for closure. A single incidence of left recurrent laryngeal nerve injury is reported by Donohoe and Gee6 via a right lateral approach. In the present case the first reoperation was required for closure of the upper cleft missed at the initial operation. The second reoperation was required to close the most proximal extent of the cleft in the supraglottic region as well as repair of three suture line fistulas. Geiger et a123and Imbrie and Doyle” each report a case in which reoperation to close the supraglottic cleft was required for resolution of symptoms. The use of nonabsorbable sutures and apposition of suture lines was responsible for the recurrent fistulas in the

two

present case. We recommend absorbable sutures and either distraction of the suture lines or interposition of soft tissue. This can be accomplished using surrounding connective tissue, strap muscle, or sternocleidomastoid.’ As in this case, the salient points in all cleft surgery are: (1) precise definition of the cleft endoscopically; (2) securing the airway and eliminating the risk of aspiration; (3) proceeding with operative repair only with optimal nutritional and pulmonary status; (4) cleft closure with absorbable suture including the interarytenoid area; (5) preventing apposition of suture lines; and (6) placement of tracheostomy, gastrostomy, and prophylactic antireflux procedure in long clefts.

REFERENCES 1. Roth B, Rose KG, Benz-Bohm G, et al: Laryngotracheoesophageal cleft-Clinical features, diagnosis, and therapy. Eur J Pediatr 140:41-46, 1983 2. Cotton RT, Myer CM, Holmes DK, et al: Laryngeal and laryngotracheoesophageal clefts: Role of early surgical repair. Ann Otol Rhino1 Laryngol99:98-104, 1990 3. Evans JNE: Management of the cleft larynx and tracheoesophageal clefts. Ann Otol Rhino1 Laryngol94:627-630, 1985 4. Glossop LP, Smith RJH, Evans JNG: Posterior laryngeal cleft: An analysis of ten cases. Int J Pediatr Otol Rhino1 Laryngol 7:133-143.1984 5. Ogawa T, Yamataka A, Miyano T, et al: Treatment of laryngotracheoesophageal cleft. J Pediatr Surg 24:341-342,1989 6. Donahoe PK, Gee PE: Complete laryngotracheoesophageal cleft: Management and repair. J Pediatr Surg 19:143-148,1984 7. Hof E, Hirsig J, Giedion A, et al: Deleterious consequences of gastresophageal reflux in cleft larynx surgery. J Pediatr Surg 22:197-199, 1987 8. Benjamin B, Inglis A: Minor congenital laryngeal clefts: Diagnosis and classification. Ann Otol Rhino1 Laryngol 98:417420,1989 9. Armitage EN: Laryngotracheo-oesophageal cleft-A report of three cases. Anesthesia 39:706-713,1984 10. Jorgensen K, Godballe C, Sorensen JA, et al: Congenital laryngo-trachea-esophageal cleft: Report of a case and difficulties of diagnosis and treatment. Acta Otolaryngo1449:105-108, 1988 11. Tucker GF, Maddalozzo J: Occult posterior laryngeal cleft. Laryngoscope 97:701-704,1987 12. Kauten SR, Konrad HR, Wichterman KA: Laryngotracheoesophageal cleft in a newborn infant. Curr Surg 41:101-104, 1984 13. Holinger LD, Tansek KM, Tucker GF: Cleft larynx with airway obstruction. Ann Otol Rhino1 Laryngo194:622-626,198s 14. Cohen SR: Posterior cleft larynx associated with hamartoma. Ann Otol Rhino1 Laryngol93:443-446, 1984 15. Chitwood WR, Bost WS, Pories WJ, et al: Laryngotracheoe-

sophageal cleft: Endoscopic diagnosis and surgical repair. Ann Thorac Surg 48:292-294,1989 16. Dubois JJ, Pokorny WT. Harberg FJ, et al: Current management of laryngeal and laryngotracheoesophageal clefts. J Pediatr Surg 25:855-860,199O 17. Tyler DC: Laryngeal cleft: Report of eight patients and a review of the literature. Am J Med Genet 21:61-75, 1985 18. Wolfson PJ, Schloss MD, Guttman FM: Laryngotrachoesophageal cleft: An easily missed malformation. Arch Surg 119:226-230, 1984 19. Petersen G: Inhibited separation of the larynx and the upper part of the trachea form the esophagus in a newborn: Report of a case successfully operated upon. Acta Chir Stand 10:250-255,1955 20. Fuzesi K, Young DG: Congenital laryngotracheoesophageal cleft. J Pediatr Surg 11:933-937, 1976 21. Jahrsdoerfer RA, Kirchner JA, Thaler SU: Cleft larynx. Arch Otolaryngol86:108-113,1967 22. Bell DW, Christiansen TA, Smith TE, et al: NO TITLE Ann Otol86:616-622,1977 23. Geiger JP, O’Connor TJ, Carter SC, et al: Laryngotrachealesophageal cleft. J Thor Cardiovasc Surg 59:330-334, 1970 24. Friedman M, Torium DM, Grybauskas V, et al: Nonrecurrent laryngeal nerves and their clinical significance. Laryngoscope 96:87-90,1986 25. Calceterra TC, McClure R, Ward PH: Effect of laryngofissure of the developing canine larynx. Ann Otol83:810-813, 1974 26. Montgomery WW, Smith SA: Congenital Laryngeal defects in the adult. Ann Otol85:491-497,1976 27. Imbrie JD, Doyle PJ: Laryngotrachoesophageal cleftReport of a case and review of the literature. Laryngoscope 79~252-274,1969 28. Hendren WH: Repair of laryngotrachoesophageal cleft using interposition of a strap muscle. J Pediatr Surg 11:425-429, 1976

Discussion P. Donahoe (Boston, MA): In the management of complex reoperative cases it is wise to have a broad armamentarium of surgical techniques to attack the

problem. Therefore, it would be inappropriate to be rigid in a choice of the anterior fissure or the lateral approach. Our theoretic hestiation in the use of the

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anterior fissure approach is that it may further weaken the trachea; therefore, we prefer the lateral appreach, but have had occasion to use the anterior approach. The lateral approach permits interposition of the medial head of the sternocleidomastoid, which

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we have found to be extraordinarily helpful in these lesions. These children often will present after multiple repairs so that in many cases the choice of the anterior approach will be as it was in this child, quite appropriate, as the excellent end result attests.

Operative strategy for recurrent laryngeal cleft: a case report and review of the literature.

A case is reported of recurrent laryngotracheoesophageal cleft following two attempted repairs. The anterior approach to the larynx and trachea provid...
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