Ltd. All rights
Tracheoesophageal W. Gregory Departmenr
Ann K. White and Robert
fistula: A case report
of Western Onlario.
(Received 7 April 1992) version received 5 November (Accepted 9 November 1992)
N6A 4 V.? i Cm&i
Tracheoesophageal fistula without atresia (H-type) is a known but uncommon cause of respiratory distress in the newborn. We present a rare case of a high cervical tracheoesophageal fistula unassociated with a laryngeal cleft or esophageal atresia. The diagnosis, associated anomalies, and surgical repair of this previously undescribed entity are discussed.
Key words: Tracheoesophageal
The incidence of anomalies involving the tracheoesophageal complex is l/3000-4500 births. The etiology is not known. Related teratogenesis has implicated both genetic as well as environmental factors . Associated congenital anomalies occur in over half of these patients [l]. Tracheoesophageal fistula without atresia (H-type) is an uncommon anomaly within the complex of esophageal atresia (EA) and/or tracheoesophageal fistula (TEF), accounting for less than 5% of TEF presentations.
The patient is a 9-day-old infant girl who had symptoms of dysphagia and aspiration with feedings. The child is a 42 week gestation female born to a 33-year-old Correspondence
Ann K. White, Atlanta Ear, Nose Suite 201, Atlanta, Georgia 30342, USA.
G2P2002 mother. Pregnancy and labor were uneventful. Birth weight was 3150 g with Apgar scores of 8 at 1 min and 9 at 5 min. The child showed no dysmorphism but ambiguous genitalia and an abnormal cystic structure arising from the region of the urethra was evident (Fig. 1). Urine was noted in the vagina. Early on, breast feeding resulted in choking episodes associated with cyanosis, tachypnea and indrawing. Chest X-ray demonstrated no aspiration; however, multiple vertebral anomalies were seen. These include 13 pairs of ribs, one accessory left rib at the T9 level, a left thoracic hemivertebra, fusion of thoracolumbar vertebrae and an abnormal configuration of the sacrum (Figs. 2 and 3). A small ventricular septum defect was also diagnosed. Subsequent nasogastric tube feedings resulted in further ‘dusky’ spells, regurgitation, tachypnea, indrawing and chest rhonchi. Despite these problems, the child maintained oxygen saturations of 84-95% on room air.
Fig. 1. The abnormal genitalia: note the large cystic structure arising from the urethra.
Fig. 2. Radiograph demonstrating 13 pairs of ribs, one accessory rib, a left hemivertebra and abnormal sacrum.
Evaluation of the child’s dysphagia by tine-esophagram demonstrated laryngeal penetration and aspiration of contrast material, possibly consistent with a laryngeal cleft or esophageal web (Figs. 4 and 5). On our examination, the child was tachypneic at 60-80 breaths/min and had mild nasal flaring and indrawing. Stridor was absent. Auscultation of the chest revealed
Fig. 3. Lateral radiograph demonstrating fusion of the thoracolumbar vertebrae.
diffuse rhonchi. There were no abnormalities of the oral cavity, oropharynx, or neck. The cry was strong. Diagnostic laryngoscopy and bronchoscopy confirmed the presence of an H-type tistula located just below the inferior edge of the cricoid cartilage in the right posterolateral aspect of the subglottis. The fistula was widely patent. The remainder of the tracheobronchial tree and larynx was normal. There was no laryngeal cleft or esophageal web present.
Fig. 4. Lateral
view from preoperative
in the main bronchi,
the right main bronchus
Transcervical repair of the fistula was carried out via a right horizontal skin incision at the level of the cricoid cartilage. With the great vessels retracted laterally and the larynx rotated medially, the recurrent laryngeal nerve was identified and preserved. The previously inserted rigid Storz bronchoscope was positioned at the opening of the fistula. The tistulous tract was then identified in the neck extending from the level of the cricopharyngeus muscle to just inferior to the cricoid cartilage (Fig. 6).
Fig. 5. Frontal view from preoperative tine-esophagram shows contrast in the main bronchi, especially the right main bronchus.
The diameter of the fistula appeared at least 50% of the diameter of the normal esophagus. The fistulous tract was ligated using two 3-O silk ties; it was not divided. The subglottis was forcefully insufflated using the bronchoscope at the opening of the tistula but no air was noted to enter the esophagus after ligation, The wound was closed in layers over a small penrose drain. Intravenous dexamethasone was used for 36 h postoperatively to decrease laryngeal edema. Following self extubation on postoperative day 1, mild stridor
- Inferior constrictor
lumen (Shaded )
Tracheal lumen (Shaded)
Fig. 6. Line diagram depicting the position and location of the tistula
resolved but tachypnea persisted. Nutrition was supplied by peripheral hyperalimentation. Fiberoptic examination of the larynx on postoperative day 12 revealed a persistent right true vocal cord palsy with good compensation by the left true vocal cord. The subsequent swallowing study demonstrated no aspiration and no regurgitation of contrast material (Fig. 7). Persistent postoperative tachypnea was traced to problems of mild congestive heart failure secondary to the child’s ventriculoseptal defect (VSD) and responded to digoxin. Oral feedings progressed rapidly and the child was discharged on the 22nd postoperative day. Discussion Tracheoesophageal fistulas result from incomplete development of the tracheoesophageal septum. TEF without atresia embryologically can occur anywhere from the cricoid cartilage to the carina. The majority of reports of this condition have described the tistula at the level of the second thoracic vertebra or in the ‘low’ cervical region [7,11- 131, This is the first case of our knowledge of the tistula arising immediately below the substance of the cricoid cartilage, taking a cephalad course to enter the esophagus at the level between the interdigitating fibers of the cricothyroid and cricopharyngeus muscles.
Fig. 7. Frontal view from postoperative tine-esophagram demonstrating fistulous tract.
no contrast media entering
The isolated TEF can be subtle in its presentation and often presents a diagnostic challenge. The diagnosis is easily missed in the neonatal period [I 1. Symptoms typically start from birth. Feeding is associated with coughing and cyanotic spells. Liquids cause more symptoms than solids. Pneumonia resistant to therapy is common. Abdominal distention associated with belching and flatus is often present 17). A description of a ‘mucousy’ child by the caretakers should also alert the surgeon to the diagnosis of H-type fistula .
Radiological evaluation using cineflouroscopy may demonstrate a fistula which is not seen with an ordinary barium swallow . Positioning the patient in the prone Trendelenburg position during catheter insertion optimizes demonstration of the fistula, due to the ventral and cephalad direction of most fistulous tracts . Benjamin has outlined the importance of endoscopy in both the diagnosis and treatment of H-type fistulas . Careful inspection of the membranous trachea will usually demonstrate the fistula if present. The opening is seen in the posterior wall, usually in the midline or to the left of the midline. This isthe first case to our knowledge of the opening appearing to the right of the midline. The combination of cineradiography and endoscopy can accurately determine the level of the fistulous tract. This factor will decide the operative approach to be used. Treatment The treatment of an H-type TEF is surgical. The decision to employ a cervical vs. a thoracic approach is based upon the distance of the fistula from the vocal cords or carina. These approaches have been well documented in the literature [2,4-7,9,10,13,15]. If the fistula is at the level of T2 or higher, a cervical approach is used. If at T3 or lower, a thoracic retropleural approach gives better exposure. The identification of the fistula is facilitated by the introduction of the rigid bronchoscope in order to illuminate the opening into the trachea. Positive pressure insufflation can be used to expand the fistulous tract, further aiding in its identification. This localizes the esophageal entrance and prevents unnecessary dissection which may damage the recurrent laryngeal nerve. Once the tract is identified, two heavy, non-absorbable ligatures are used to occlude the fistulous tract. The success of the ligature can be checked by using positive pressure insufflation through the bronchoscope. No further inflation of the tract suggests a successful ligature. A layered closure of the cervical incision over a penrose drain is used. Postoperatively, intravenous dexamethasone is used to decrease laryngeal edema. Nutrition is supplied by peripheral hyperalimentation or gavage feedings. Repeat cineradiofluroscopy is used to document the success of the procedure. Fiberoptic laryngeal examination should be performed to document the function of the vocal cords. Associated anomalies Associated anomalies are present in half the patients with TEF and atresia. One third of patients with H-type fistulas have associated anomalies. A pattern of anomalies referred to as the VATER association has been described . This includes vertebral defects, anal atresia, TEF with or without atresia, radial and renal dysgenesis. The VACTERL acronym was used by Manning to include cardiovascular anomalies and limb defects [lo]. Numerous other minor anomalies have been described [7,14,16,17]. This case illustrates cardiovascular anomalies (VSD), somite formation anomalies (skeletal findings), as well as genitourinary anomalies (ambiguous genitalia, subclitoral cyst, failure of urogenital septum formation). With regards to embryogenesis this suggests a period from the 4th to 8th weeks as a common sensitivity to injury period.
Conclusion We have presented a case of an H-type tracheoesophageal fistula without atresia located at the laryngotracheal border (i.e., just inferior to the cricoid cartilage). The fistulous tract took a cephalad course to enter the esophagus at the level of the cricopharyngeus muscle to the right of the midline. To our knowledge, this is the first such presentation. This patient was treated with ligation of the tract without division, via a cervical approach. The case also illustrates associated cardiovascular, skeletal and genitourinary anomalies suggestive of a common gestational insult simultaneously affecting these developing structures.
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