ORIGINAL ARTICLE Does the Presence of a Tracheoesophageal Fistula Predict the Outcome of Laryngeal Cleft Repair?

David L. Walner, MD; Yoram Stern, MD; Michael Collins, BS; Robin T. Cotton, MD; Charles M. Myer III, MD

Objective: To determine if the presence of a tracheo- Results: Twenty-five patients were reviewed for study esophageal fistula (TEF) alters outcome following laryn- purposes. Fourteen had a history of TEF repair and 11, geal cleft repair. no history of TEF. All 25 patients underwent surgical re- pair of the laryngeal cleft. Twelve of the 14 patients with Design: A retrospective review of patients diagnosed and a history of TEF repair experienced a breakdown of the treated for laryngeal clefts, with a minimum follow-up laryngeal cleft repair. Only 1 of the 11 patients with no period of 1 year. history of TEF experienced such a breakdown. In 8 of 9 patients with a laryngotracheoesophageal type I cleft, sur- Setting: An academic tertiary care children’s hospital. gical repair was not successful.

Patients: Twenty-five pediatric patients diagnosed and Conclusions: In our series, patients with laryngeal clefts surgically treated for laryngeal cleft. who also had a history of TEF had a much higher inci- dence of breakdown of cleft repair compared with pa- Main Outcome Measures: Each chart was tients with no history of TEF. This finding is not conclu- reviewed to determine if patients with a laryngeal cleft sive and requires further investigation. The failure of cleft had been diagnosed with TEF and had undergone a repair correlated with the severity of the cleft. The impor- surgical TEF repair procedure. The success of the tance of these associations may lead to enhanced surgical surgery was evaluated based on the resolution of planning and realistic preoperative family expectations. symptoms and the endoscopic evaluation of the repair site. Arch Otolaryngol Head Neck Surg. 1999;125:782-784

ARYNGEAL CLEFTS (LCs) and A major difficulty in discussing the sur- laryngotracheoesophageal gical options and results in patients with clefts (LTECs) are rare con- airway clefts is the multiple classification genital anomalies. The ap- schemes and confusing nomenclature used. proximate incidence is 1 in Classification schemes have been described 10L 000 to 20 000 live births.1 by Pettersson, Armitage, Benjamin, Evans, Multiple congenital anomalies can be Myer, and others. To discuss surgical tech- associated with LC and LTEC. These in- nique or surgical outcome, one must com- clude tracheoesophageal fistulas (TEFs), pare clefts involving the same anatomical re- laryngomalacia, cleft lip and palate, sub- gions. For this reason, we use the Myer- glottic stenosis, hypoplastic lungs, trans- Cotton5 scheme, outlined below, which is position of the great vessels, hamartoma, anatomically specific and descriptive. From the Department of imperforate anus, rectal stenosis, mesen- Cleft Location Cleft Type Otolaryngology/ teric malrotation, Meckel diverticulum, hy- Laryngeal 2 Bronchoesophagology, pospadias, and hypoplastic kidneys. Interarytenoid L I Rush-Presbyterian-St Luke’s Tracheoesophageal fistulas have been Partial cricoid L II Medical Center, Chicago, and identified in 20% to 37% of patients with Complete cricoid L III Lutheran General Children’s LC and LTEC.3,4 Two senior authors Laryngotracheoesophageal Hospital, Park Ridge, Ill (R.T.C. and C.M.M.) have observed that Into trachea LTE I To carina LTE II (Dr Walner); and Department a much higher incidence of failed LC and of Otolaryngology and LTEC repairs can be found in patients This system differs from most others by Maxillofacial Surgery, Children’s Hospital Medical with a history of TEF. The purpose of dividing the clefts into LCs and LTECs Center, Cincinnati, Ohio this study was to specifically compare rather than attempting to use one term to (Drs Walner, Stern, Cotton, the surgical outcome of LC and LTEC describe all variations. Each of these is then and Myer and repair in patients with and without a his- subdivided to give specific information as Mr Collins). tory of TEF. to the location of the cleft.

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©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 Type of Cleft Recurrences Based on the Myer-Cotton5 PATIENTS AND METHODS Classification System*

Cleft Type TEF Group Non-TEF Group We performed a retrospective review of patients di- LI 0 0 agnosed and treated for LC and LTEC between Janu- L II 3 (2/3 recurred) 5 (0/5 recurred) ary 1985 and December 1996 at Children’s Hospital L III 3 (3/3 recurred) 5 (0/5 recurred) Medical Center, Cincinnati, Ohio. Inclusion in our re- LTE I 8 (7/8 recurred) 1 (1/1 recurred) view required a minimum of 1-year follow-up from LTE II 0 0 the time of surgical repair. Patients with L I– or LTE II–type clefts were excluded from the study. All charts *TEF indicates tracheoesophageal fistula; L, laryngeal; were reviewed and data collected regarding age at the and LTE, laryngotracheoesophageal. time of cleft repair, the presenting symptoms, the type of cleft present (based on the Myer-Cotton classifica- laryngotomy. Two patients had undergone initial repair tion system), any associated anomalies or comorbid conditions, the type of cleft repair performed, and the at other institutions for which a lateral pharyngotomy was postoperative status with regard to the formation of a used. In 8 of 9 patients with LTE I clefts, breakdown of recurrent cleft or an intact surgical repair site. the surgical repair was identified.

COMMENT Laryngeal clefts and LTECs are uncommon congenital RESULTS anomalies. Many associated conditions can coexist, in- cluding congenital heart disease, gastrointestinal and gas- Twenty-five patients’ charts were available for review. The trourinary tract abnormalities, and midline defects. The average age at the time of surgery was 2.4 years (age range, condition is thought to be familial, with an autosomal 2 weeks to 9 years). The most common presenting symp- dominant inheritance pattern.6 In addition, LC and LTEC toms were feeding problems, aspiration, stridor, and cya- are identified as part of 2 syndromes: the Opitz-Frias syn- notic episodes. The associated anomalies and comorbidi- drome, or G syndrome, consists of hypertelorism, cleft ties are listed below. lip, cleft palate, airway cleft, and hypospadias; the Pallister- Anomalies and/or Comorbidities Patients, No. (%) Hall syndrome consists of congenital hypothalamic Tracheomalacia 15 (60) hamartoblastoma, hypopituitarism, airway cleft, imper- Esophageal atresia 7 (28) forate anus, and postaxial polydactyly.7 Subglottic stenosis 6 (24) Pettersson8 performed the first surgical repair of an Bronchomalacia 5 (20) Congenital heart disease 4 (16) LC in 1955. Postsurgical success of LCs depends on main- Opitz syndrome 4 (16) taining an intact wall separating the airway and the di- 4 Prematurity 4 (16) gestive tract. Evans et al described 15 patients who had Hypospadias 4 (16) open repairs of LCs or LTECs, 4 (26.7%) of which re- Anal stenosis 3 (12) quired revision. Two of the repairs required multiple re- Polyhydramnios 3 (12) visions, and 1 patient died prior to revision surgery. Other Vater syndrome 2 (8) studies have reported anastomotic leaks in as many as Malrotation of gut 2 (8) 50% of postoperative patients.9 A review of 170 clefts re- Bronchopulmonary dysplasia 2 (8) ported prior to 1991 identified only 19 (11.2%) that re- Fifteen of the patients required preoperative feeding tubes, quired revision.10 Most studies, including ours, have con- and 11 underwent preoperative Nissen fundoplications. cluded that the incidence of revision surgery increases Twenty patients had tracheostomy tubes placed preop- with the severity of the cleft.4,5 eratively, and 14 were decannulated at some point in the Treatment options depend on the classification of first year following surgery. Six patients required a la- the cleft. Type L I clefts may be treated in some cases with ryngotracheoplasty with costal cartilage grafting prior to antireflux therapy alone. If still symptomatic, these pa- eventual decannulation. Three patients had redundant tients may need an endoscopic or open surgical repair. mucosa in the posterior aspect of the airway following Types L II, L III, and LTE 1 are best treated using an an- repair; 2 of these required laser excision of the mucosa. terior laryngotomy. Type LTE II requires a lateral phar- Two patients died approximately 1 year following sur- yngotomy. Most patients will require a tracheostomy ei- gery, only 1 whom had an intact surgery repair. ther preoperatively or perioperatively. For shorter- Fourteen patients had a history of TEF and had un- length clefts that require only minimal airway dergone TEF repair prior to the LC or LTEC surgery. manipulation, a single-stage approach may be reason- Twelve (86%) of these patients developed a recurrent LC able. For more extensive clefts, a tracheostomy will likely or LTEC. Seven of these patients required multiple (Ͼ3) be necessary for airway control and protection. procedures in the attempt to repair the cleft. Our study found a much higher incidence of break- Eleven patients had no history of TEF. One (9%) of down of cleft repair in patients with a history of TEF. these patients developed a recurrent LC or LTEC. Though most of these patients had undergone their ini- Cleft types are listed in the Table. All cleft proce- tial repair at other institutions, the referral pattern ap- dures (LC and LTEC) were performed using an anterior peared to be spread equally throughout the country. The

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©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 explanation for this breakdown cannot be ascertained from cosa and estimating the amount of esophageal mucosa to the retrospective nature of this study. However, possible preserve once the cleft site is exposed transcervically. explanations include impaired wound healing or break- Our study excluded patients with type L I clefts be- down of cleft repair due to impaired esophageal motility. cause many of these do not require surgery. When sur- It is well known that patients with TEF have esophageal gery is required, it is controversial whether the ap- dysmotility.11 This leads to stasis of secretions and food proach should be endoscopic or open. We also excluded material at the surgical repair site, which cause an inflam- type LTE II clefts because these are often acutely life threat- matory reaction and wound breakdown. ening and involve an extensive care and decision mak- The most common type of TEF is esophageal atre- ing process. Discussion of these 2 excluded groups is be- sia with a distal TEF, the esophageal gap usually being 2 yond the scope of this article. cm in length and the fistula most often occurring at the RecurrentLTECsarethoughttooccurmorecommonly carina area. This type of defect is found in 85% of pa- when the primary repair used nonabsorbable suture ma- tients with TEF.11 The H type of TEF accounts for only terial and when the suture lines are in direct apposition.10 3% to 5% of all TEFs.11 In our 14 patients with TEF, only The use of muscle interposition with the sternohyoid muscle 7 had esophageal atresia. In other studies on patients with wasdescribedin1976totreat2recurrentclefts.13 Techniques TEF, the incidence of LC or LTEC is only about 6%.9 have also been described using other material for interpo- InpatientswithbothLTECandTEF,cine-esophagrams sition. We use muscle interposition to enhance the integ- may show rapid “spillover” into the trachea with less than rity of the surgical repair for primary cases of poor-quality 1 mL of contrast material.12 Endoscopy remains the main- tissue and for recurrent cleft repairs. stay in diagnosing LTEC. This should consist of a micro- Our intention is to make pediatric otolaryngolo- laryngoscopy, bronchoscopy, and esophagoscopy. Often gists and surgeons aware of the poor outcome of LC and an anterior commissure–type laryngoscope is helpful, along LTEC repair in patients with a history of TEF. This in- with a suspension apparatus to provide the best visualiza- formation may lead us to using muscle interposition at tion of the posterior laryngeal and tracheal region. The con- the time of primary cleft repair in patients with TEF. Close dition involves infolding of tissue in the posterior aspect follow-up is essential in the TEF group of patients fol- of the airway, which must be splayed to its maximum width lowing cleft repair. Preoperative parental meetings need to fully evaluate the cleft. It is essential to identify the length to address the issue of follow-up and potential break- of the cleft and its relationship to key structures, includ- down of cleft repair. ing the true vocal folds, the cricoid cartilage, and the cari- na. A narrow flexible ureteric catheter can be used to pal- Accepted for publication February 26, 1999. patetheposteriortrachealwallandprobepreviouslyrepaired Presented at the Thirteenth Annual Meeting of the TEF sites to ensure that they are intact. It is not uncommon American Society of Pediatric Otolaryngology, Palm Beach, to find a small pouch at the TEF repair site, which may have Fla, May 14, 1998. a more funicular shape in a patient with LTEC than in one Reprints: David L. Walner, MD, Rush-Presbyte- without a cleft.9 rian-St Luke’s Medical Center, Department of Otolaryn- A stable airway must be established for each pa- gology/Bronchoesophagology, 1653 W Congress Pkwy, tient and aspiration prevented. Chronic and irreversible Chicago, IL 60612. pulmonary injury can occur from a few weeks of aspi- ration, especially in the neonate. Gastroesophageal re- flux must be controlled prior to surgery to optimize the REFERENCES cleft repair. 1. Roth B, Rose KG, Benz-Bohm G, Gunther H. Laryngo-tracheo-esophageal cleft: We believe that the anterior laryngotomy is best for clinical features, diagnosis, and therapy. Eur J Pediatr. 1983;140:41-46. all type L II, L III, and LTE I clefts. This approach offers 2. 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