Anterior cervical approach for repair of laryngotracheoesophageal cleft

Anterior Cervical Approach of Laryngotracheoesophageal By G.S. tipshutz,

CT. Albanese, San

Francisco,

Laryngotracheoesophageal cleft is a very rare congenital malformation characterized by a long midline communication between the esophagus and trachea extending from the larynx down the trachea for a variable distance. The most common repair strategy involves separation of the trachea and esophagus by a lateral approach via both a thoracotomy and a neck incision. The authors report a case of complete

L

ARYNGOTRACHEOESOPHAGEAL clefts are rare anomalies resulting from the failure of normal separation of the esophagus and trachea. The presence of a common proximal aerodigestive channel coupled with the common association of gastroesophageal reflux and microgastria allows aspiration and respiratory embarrassment that leads to a high mortality rate. Laryngotracheoesophageal clefts are classified according to the length of the common aerodigestive channel: type I clefts involve the supraglottic larynx, type II clefts extend beyond the cricoid to the cervical trachea, type III clefts involve the entire trachea, and type IV clefts extend to one or both mainstem bronchi.lm3 Repair of the severe, distal clefts involves separation of the trachea and esophagus by lateral approach through both a thoracotomy and neck incision. Such an approach potentially compromises blood supply to both the esophagus and trachea, and risks injury to the recurrent laryngeal nerves. The present report describes a novel, anterior cervical approach to the transtracheal repair (without thoracotomy) of the rare type III cleft. CASE

M.R. Harrison,

repair (without thoracotomy) anterior wall of the trachea. J Pediatr Surg 33:400-402. ders Company. INDEX foregut

WORDS: anomalies,

via

a neck

Copyright

incision o 1998

Laryngotracheoesophageal laryngeal cleft.

through by W.B.

cleft,

the Saun-

congenital

respiratory status progressively deteriorated on mechanical ventilatory support, Breath sounds were detected over both the lung fields and the stomach. Flexible bronchoscopy demonstrated a type III laryngotracheoesophageal cleft. Figure 1 demonstrates the cleft that extends from the arytenoids to 1 cm above the carina. The patient was transferred to our institution for immediate surgical management. The infant was positioned with the neck maximally extended. Because the stomach was distended from the positive pressure of mechanical ventilation through the cleft, a gastrostomy tube was placed to decompress the stomach and a fundoplication performed. Next, a low transverse neck incision with a lower midline “T” extension and upper median sternotomy through the manubrium allowed the trachea to be exposed from the larynx to the carina. The endotracheal tube was then advanced and “wedged” in the distal 1 cm of normal trachea adjacent to the carina. The trachea was divided along the anterior midline from the larynx to the carina (Fig 2). The mainstem bronchi were then alternately intubated across the surgical field, allowing clear visualization of the back wall of the common channel. A nasogastric tube was placed and was clearly visualized in the operative field. The esophagus was then separated from the trachea by incising from the inside (Fig 3). Unequal flaps were created so the esophageal and tracheal anastomoses were not superimposed. The esophagus was closed with interrupted 4-O polyglactin suture with knots on the inside (Fig 4) followed by tracheal closure in a similar fashion (Fig 5). The suture line overlapped only at the very proximal and distal ends of the repair. A custom fabricated 90” tracheostomy tube was

REPORT

From The Fetal Trentment Centel; University of California, San Francisco, CA. Presented at the 28th Annual Meeting of the American Pediatric Sur~icalAssoci&ion,, Naples, Florida, May 18-21, 1997. Address reprint requests to Russell W Jennings, MD, The Fetal Treatment Center; University of California, San Francisco, 3rd and Parnassus Aves, Rm I601 HSW, San Francisco, CA 94143. Copyright 0 I998 by WB. Saunders Company

400

and R.W. Jennings

California

A karyotypically normal 1,950-g boy was born by spontaneous vaginal delivery at 34 4/7 weeks. The pregnancy was complicated by preeclampsia and polyhydramnios. Although the Apgar scores were 7 at 1 minute and 8 at 5 minutes, progressive respiratory compromise ensued requiring emergency endotracheal intubation. The patient’s

0022-3468/98/3302-0049$03.00/O

for Repair Cleft

Normal

Cl&

Fig 1. The type III laryngotracheoesophageal cleft extends down to the carina. A ccmm~n tracheoesophageal channel is present with the trachea anterior and the esophagus posterior.

Journal

of Pediatric

Surgery,

Vol33,

No 2 (February),

1998: pp 400.402

LARYNGOTRACHEOESOPHAGEAL

Fig 2. The surgical approach involves a long midline incision through the anterior surface of the trachea exposing the posterior tracheal cleft. The distal airway must be intubated across the operating field.

placed. The patient remained mechanically ventilated and was transferred to the intensive care nursery. The postoperative course was complicated by prolonged mechanical ventilatory support because of posterior tracheomalacia and bronchomalacia. Vocal cord motion was normal postoperatively. Three weeks postoperatively, an upper tracheoesophageal fistula, caused by posterior erosion of the tracheostomy tube, was diagnosed. It was repaired via a left lateral neck approach, interposing a sternocleidomastoid muscle flap between the trachea and esophagus. A left vocal cord palsy was diagnosed after this procedure but did not prevent extubation. After several laser ablations of granulation tissue, the tracheostomy tube was removed 8 weeks postoperatively. An esophageal stricture formed at the site of fistula repair, which responded to dilation therapy. On discharge at 4 months of age, the infant was swallowing his own secretions and required no supplemental oxygen. He eats by gastrostomy tube because of feeding aversion but appears to be otherwise normal developmentally. Serial endoscopic findings have shown only mild posterior tracheomalacia and right mainstem bronchomalacia and no further granulation tissue. The anterior tracheal closure and esophagus are well healed.

DISCUSSION

Laryngotracheoesophageal clefts are uncommon and are difficult to diagnose early. The presence of a laryngotracheoesophageal cleft should be suspected in an infant

Fig 3. To avoid overlapping recurrent tracheoesophageal the trachea is separated from

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CLEFT

suture lines and to minimize the risk of fistula, unequal flaps are created when within the esophagus.

Fig 4. The esophagus is tubularized using interrupted absorbable sutures with the knots on the inside. The posterior wall of the trachea is then repaired in a similar fashion.

with a hoarse cry, stridor, and aspiration pneumonia.4-8 In addition, an endotracheal tube that appears to migrate into the esophagus,9 or an esophageal tube that appears to become displaced into the trachea may also be clues to a laryngotracheoesophageal cleft. Definitive diagnosis is made during endoscopy,8 but may be suggested during an esophageal contrast study. Endoscopically, the cleft can easily be missed because it is narrow, and the edges tend to coapt from the pressure of the tracheal rings. Once diagnosed, definitive surgical repair should be quickly undertaken because life-threatening pulmonary complications may develop, especially with long clefts.2J0 The type of cleft directs the operative technique. Type I defects can be repaired endoscopically, without a tracheostomy.1° Type II clefts are often repaired through the anterior wall of the larynx and trachea, providing excellent exposure without risk to important neurovascular structures.l” Type III and IV clefts can be associated with other congenital anomalies including esophageal atresia, microgastria, and congenital heart disease”-I4 as part of the VACTERL association. The logical and common

Fig 5. absorbable position.

The anterior midline trachea is repaired with interrupted sutures and the 90” tracheostomy tube is secured in

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LIPSHUTZ

approach to repair of the type III and IV laryngotracheoesophageal clefts is laterally, through a right thoracotomy and right neck incision.9 In contrast to the lateral repair, we chose to use a midline transtracheal exposure (as is often done for type II laryngeal cleft repairs and short type III laryngotracheoesophageal cleft repairs*) to avoid recurrent laryngeal nerve injury and possible devascularization of the trachea and esophagus. We believe this approach is safer than the lateral approach, although it is technically more demanding, and it adds a third suture line. The present report illustrates several important principles in the management of long laryngotracheoesopha-

ET AL

geal clefts. The anterior approach is safe and technically feasible for the long cleft. Intraoperative airway control with an open trachea is performed with intubation across the operative field, similar to techniques used during repair of tracheal stenosis. A tracheoesophageal fistula can occur despite meticulous attempts to avoid overlapping suture lines, most likely because of the tracheostomy tube. Thus, one may consider endotracheal intubation only postoperatively, although prolonged intubation may endanger the reconstructed larynx. Prolonged postoperative mechanical ventilation may be necessary because of tracheomalacia and bronchomalacia, not secondary to an anatomic abnormality or vocal cord paresis.

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