The incidence of subglottic stenosis is reported to be on the increase because of the survival of
                     many more premature infants than in the past. About 12 per cent of upper airway obstructions
                     are from this problem. Often laryngomalacia is thought to be the problem when it is really
                     subglottic stenosis. Croup is another misdiagnosis. The primary problem is that there is a
                     thickening of the soft tissue of the tracheal wall from the vocal cords to the cricoid cartilage. The
                     thickening can be entirely circumferential of partially and usually is greatest 2 to 3 mm below the
                     vocal cords. You will hear that the most severe cases of subglottic stenosis require an anterior
                     cricoid split operation in which the cricoid cartilage is divided to alleviate as much of the
                     narrowing as possible. If the split is unsuccessful, the next step is a laryngoplasty or a complete
                     laryngeal reconstruction.

                     In Koff, on page 134, there is a photograph of a bronchoscopic view of subglottic stenosis.
                     Apparently this case was severe because a cricoid split procedure was required. Be sure to read
                     the section on tracheal stenosis on page 134.

                     Aaron Bissel is a patient we had at Children's Hospital who had subglottic stenosis. You can see
                     his picture and read about him at his website at http://members.aol.com/trachtube/aaron.htm. Or
                     go to "Link Sharing" at Nicenet and click "Aaron's Page." Aaron's surgeon was Gerald Healy,
                     who pioneered tracheal reconstruction. This little boy, although born extremely premature, has
                     done really well, thanks to the success of his surgery and the great follow-up care. He still has his
                     trach, but it is possible that he will not need it for all of his life. And Aaron's family is wonderful.
                     At this web site, you can read about tracheal reconstruction.

                     Please take a moment to go to the Electric Airway site sponsored by the Virtual Hospital
                     (http://www.vh.org/Providers/Textbooks/ElectricAirway/Videos/TrachDilBronch.mov). At this
                     site you can read about subglottic stenosis and see some endoscopic pictures. You can also get
                     to this site from Nicenet by going to "link Sharing" and clicking "electric airway."

                     In the area of the trachea, the four most common disorders that occur are tracheomalacia (which
                     you should know quite a bit about), tracheal stenosis, tracheal cysts, and tracheal atresia.
                     Tracheomalacia is a condition of both the neonatal and infant airway characterized by weakness
                     of the supporting tracheal cartilage and widening of the posterior membranous wall. Together,
                     these factors cause tracheal collapse especially during times of increased airflow, such as
                     coughing, crying, or feeding. Now imagine. The tracheal wall is floppy and flaccid in this
                     condition. Yet I have seen many a patient with this problem who can sit quietly and breathe
                     normally only to have severe distress when agitated. Tracheomalacia most commonly affects the
                     distal one-third of the trachea and can be associated with a variety of congenital anomalies
                     including cardiovascular defects, developmental delay, gastroesophageal reflux, and
                     tracheoesophageal fistula. It is, however, rarely found in combination with laryngomalacia
                     because of the separate developmental pathways for both the trachea and the larynx.

                     Although the accepted term remains "tracheomalacia," it is sometimes called "major airway
                     collapse" in some of the recent literature. There are three categories based on the histological,
                     endoscopic, and clinical presentation. Type one includes congenital or intrinsic tracheal
                     abnormalities which can be associated with a tracheoesophageal fistula. Type two involves
                     extrinsic defects or anomalies such as anomalous innominate artery, a vascular ring or a vascular
                     sling causing abnormal development or undue pressure on the trachea from a nearby blood vessel
                     (such as the aorta or, more commonly, the pulmonary artery). Type three, or acquired malacia,
                     occurs in children with prolonged intubation or chronic tracheal infections and may be a
                     progressive process. Regardless of the underlying defect or anomaly, the signs and symptoms
                     remain similar: chronic or recurrent pulmonary infections; prolonged expiratory phase; expiratory
                     stridor and coughing which often increases with feedings; and reflex apnea or "dying spells." The
                     latter are episodes of progressive hypoxia, apnea, cyanosis, bradycardia and even cardiac arrest
                     which occur during feedings. I have witnessed all of these things in children up to three years old
                     who had tracheomalacia.

                     The diagnosis of tracheomalacia (major airway collapse) depends on the patient's history
                     combined with an endoscopic evaluation (bronchoscopy). The airway must be observed during
                     spontaneous respiration which is accomplished using ventilating laryngoscopy and telescopic
                     bronchoscopy. The classic triad of laryngomalacia consists of 1) loss of the normal semicircular
                     shape of the tracheal lumen 2) forward ballooning of the posterior membranous wall; and 3)
                     anteroposterior narrowing of the tracheal lumen. The majority of these infants will respond to
                     conservative management of humidified air, chest physical therapy, or CPAP. The symptoms
                     often resolve by the age of 18 months to 24 months.

                     The children with tracheomalacia who have received a repair for an esophageal atresia or fistula
                     -we will talk about those in more detail later - may develop "dying spells" or reflex apnea. During
                     these episodes, the child develops marked apnea, cyanosis, and bradycardia usually associated
                     with feeding. These dying spells are believed to be caused by severe hypoxia. These spells are
                     treated by providing oxygen and oxygen monitoring, repositioning during feedings, and other
                     supportive measures until the child grows.

                     We had a baby in our NICU who initially had surgery for tracheoesphageal fistula (TEF), but
                     then developed tracheomalacia. The infant, was in our NICU from the time he was one day old
                     until he was almost five months old. He was a very difficult patient because he has had several
                     "dying spells." In one of them, he became so hypoxic that the attending neonatologist emergently
                     intubated him. We extubated him a few hours later, but he had one "dying spell" after another. If
                     you hang around a pediatric hospital with a strong otolaryngology service long enough, you will
                     see such patients.

                     When conservative measures are not adequate or when reflex apnea is present, surgical
                     intervention must be considered. This includes correction of the underlying cause, such as a
                     vascular ring when present, tracheostomy and aortopexy. Although effective in some cases, a
                     tracheostomy can cause airway damage and may not support the distal trachea. Aortopexy is
                     considered the treatment of choice (in vascular cases) because it relieves external pressure on the
                     flaccid trachea. It is successful in 75% of the cases of tracheomalacia. But tracheal reconstruction
                     is necessary in extreme cases.

                     Tracheomalacia and tracheal stenosis can be the result of complication from endotracheal and
                     tracheostomy tubes or infection. They also can be the result of extramural compressions from
                     malpositioned great vessels or from vascular anomalies.

                     Tracheal stenosis may be either acquired or congenital. Acquired tracheal stenosis is far more
                     common than the congenital form. Patients present with stridor, wheezing, feeding difficulties, and
                     cyanosis. Etiologies of acquired stenoses include: Vascular rings, tracheoesophageal fistula, and
                     the result of intubation or tracheostomy.

                     In congenital tracheal stenosis there is a fixed narrowing of the tracheal lumen due to the presence
                     of a complete cartilaginous tracheal ring. The lesion can be segmental, diffuse, or funnel-shaped
                     and there can be an association with a tracheal ("pig") bronchus (the stenosis occurs distal to the
                     take-off of the bronchus). Patients with cartilaginous ring abnormalities frequently have associated
                     anomalies of the respiratory tract, esophagus, skeleton, and heart.

                     One of the newer and more promising treatments for tracheal stenosis, though palliative, is
                     balloon dilatation. The result can be symptomatic improvement and increased lumen diameter in
                     patients with congenital or acquired tracheal and bronchial stenoses. More severe cases require
                     repair.

                     Cysts in the trachea are of the same type that appear elsewhere in the upper airway.. Papillomas
                     are seen in the trachea. Tracheal atresia is extremely rare and often presents with such additional
                     severe congenital anomalies, that saving the child can be futile. But there have been some
                     reported cases of tracheal atresia in which the patient survived but only after extensive tracheal
                     reconstruction.

                     Tracheoesophageal fistula (TEF) is covered in Koff on pp. 136-137. I want to discuss the
                     tracheoesophageal anomalies in general. The incidence of TEF with esophageal atresia (EA) is 1
                     in 3000 births. Premature delivery occurs in about 30% of all cases. Associated anomalies occur
                     in 50% of cases and are the most significant cause of morbidity and mortality. Esophageal atresia
                     and TE fistulas are due to failure of recanalization of the embryonic esophagus with faulty
                     separation of the primitive foregut into the trachea and esophagus. (If you recall in your study of
                     embryology, the trachea and esophagus develop at the same time from the foregut.) So affected
                     infants are commonly premature and present with respiratory distress with feeding, drooling, and
                     recurrent pneumonia's.

                     Seventy-five to 80% of all tracheoesophageal anomalies involve atresia of the upper esophagus
                     with an accompanying fistula between the lower esophageal tube and the trachea. The upper
                     esophagus ends in a blind pouch and the fistulous connection occurs between the trachea and the
                     distal esophagus. The fistula usually is just above the carina. The fistula enables the gastric juice
                     to" reflux" into the trachea and lungs, leading to a severe chemical pneumonitis. Another source of
                     aspiration is the pooled saliva in the esophageal pouch. So you can see that while the TEF and
                     EA are bad enough, the infant is set up for even more severe problems. A drawing of this and
                     other types of TEF/EA is on page 400 of Whitaker. I want you to be familiar with the two most
                     common types.

                     The next most common problem, about 8% of the tracheoesophageal anomalies, is atresia of the
                     esophagus without any fistula attachment to the trachea. This is seen on page 400 of Whitaker,
                     figure 12-1B. This means a blind-ending upper and lower segments of the esophagus.: (Atresia
                     without fistula). In these cases the abdomen will be gasless on the abdominal x-ray (which is a
                     helpful diagnostic sign). Prenatal ultrasounds of the mother commonly demonstrate
                     polyhydramnios.

                     The "H"-Type Fistula (Whitaker, page 400, Figure 12-1C) is seen in 6% of all cases, so it is
                     quite rare. In this type of fistula, the trachea and esophagus are connected by a small fistulous
                     tract. The tract typically slopes downward from the trachea to the esophagus and therefore may
                     not be visualized on contrast studies. This lesion is often not diagnosed until late infancy or early
                     childhood when the patients present with recurrent unexplained pneumonia's. Other GI atresias
                     may be seen and should be ruled out. The fistula is typically located in the proximal esophagus. A
                     clue to its location may be absent peristalsis in region of fistula.

                     Other variations of TEF are the bronchoesophageal fistulas that usually originate from lobar or
                     segmental bronchi. These are associated with pulmonary sequestration, something we will discuss
                     later. Again, these are not too common, but we have had a number of cases referred to us here at
                     Children's Hospital from other parts of the US and from Europe.

                     There are three tell-tale signs that a newborn may have a tracheoesophageal anomaly. The first
                     and most obvious is the accumulation of secretions in the mouth. The second is sporadic or
                     continuous respiratory distress, especially during feedings. The third is repeated regurgitation of
                     feedings. Usually insertion of a nasogastric tube is attempted, but the tube often winds up in the
                     esophageal pouch. Once it is determined that the infant has one of the tracheoesophageal
                     anomalies, he should be positioned at a 30 degree angle until repaired. If these infants are
                     manually ventilated because of inadvertent extubation before they are repaired, the pressure from
                     the bag will distend the gastrointestinal tract. Bagging the patient should be avoided or performed
                     minimally until the patient can be reintubated. Surgical repair is not an emergency but should be
                     performed as soon as possible.

                     After the surgical repair, it is important not to suction the infant too deeply. We usually have the
                     surgeon give us an estimate as to where the anastomosis is and measure the suction catheter to
                     that length. Also, if the infant were to become accidentally extubated before he is ready after the
                     repair , bag/mask ventilation should be avoided if at all possible. You could add too much
                     pressure to the surgical site and damage it.

                     I have included a website under "link sharing" of a case of TEF
                     (http://pandoras-box.bgsm.edu/Pathology/PC242.html). Some pretty interesting slides are
                     included with the case.

                     When a patient is diagnosed with TEF or EA, you will find that the medical team will not rest until
                     other anomalies are ruled out. Remember, fifty percent of TEF/EA cases are associated with
                     other anomalies. Two types of multiple congenital anomalies (MCA's) are important for you to
                     know. They are important because you will undoubtedly see them when and if you work in a
                     pediatric facility or a hospital with a pediatric surgery referral service. The first MCA associated
                     with TEF/EA is VATER syndrome. Usually, you'll hear it referred to as "Vater's." The VATER
                     acronym stands for Vertebral anomalies, Anal malformation, Tracheoesophageal fistula,
                     Esophageal atresia, and Radial limb dysplasia.

                     Renal and vascular anomalies may also exist with these components of VATER syndrome. And
                     on top of that, cardiac defects such as TOF. This is why that once TEF/EA is suspected, a
                     cardiology evaluation, including an ECHO, is essential prior to surgery. But when we have these
                     additional anomalies, the VATER acronym is replaced with the more recently described
                     VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, limb). Obviously, we could talk
                     about these two syndromes and their variations, but I just want you to know what these
                     acronyms mean. Obviously, if you hear that a VATER or VACTERL patient is being admitted to
                     your ICY, you need to be concerned about the airway and cardiac issues more than the others.

                     Be sure to take a look at the discussion of TEF/EA on pages 482-486 of Barnhart's "perinatal
                     and Pediatric Respiratory Care." This is an excellent passage, and the x-rays on pp. 484 and 485
                     are very representative of the cases we see frequently.