Subglottic Cysts in the James D.
Premature Infant
Smith, MD; Robin Cotton, MD; Charles M. Meyer III, MD
\s=b\ Supraglottic
cysts in the newborn
are
well-recognized entity, but subglottic cysts have been rarely reported. Over the past 6 years we have observed subglottic cysts in nine patients with relatively long a
intubations from the neonatal intensive care unit of two university hospitals. Most frequently the patients were extubated and did well for weeks or months, but then they had progressive biphasic stridor. On endoscopy the patients had a subglottic stenosis that was irregular but with a smooth mucosal lining. Usually the cysts were apparent, but in two patients the mucosa was thickened and the patients were treated as a subglottic stenosis with tracheostomy. These subglottic cysts were recognized at the time of laryngotracheoplasty. In six patients the cysts were managed either by marsupialization with cup forceps, endoscopic diathermy, or carbon dioxide laser without recurrence. It is our belief that this condition is most likely due to scarring and obstruction of mucus glands of the subglottic area from prolonged intubation. This entity should be recognized and looked for in the neonate who has an acquired subglottic stenosis and should first be treated conservatively with endoscopic marsupialization. (Arch Otolaryngol Head Neck Surg.
1990;116:479-482)
Although supraglottic cysts
and
laryngoceles are frequently rec¬ ognized, subglottic cysts have been rarely reported.16·8 Over the past 6 -ti-
years, in two separate neonatal inten¬
sive care units, we have had nine cases of subglottic mucus retention cysts in premature infants who had been intubated for varying lengths of time. REPORT OF CASES The following cases of subglottic cysts have been diagnosed at the Children's Hos¬ pital Medical Center, Cincinnati, Ohio, and
Accepted for publication September 19, 1989. From the Department of Otolaryngology\p=n-\Head and Neck Surgery, Oregon Health Sciences University, Portland (Dr Smith); and Department of Otolaryngology\p=n-\Maxillofacial Surgery, University of Cincinnati, Ohio (Drs Cotton and Meyer). Reprints not available.
Oregon Health Sciences University, Portland, from 1982 to 1988.
the
Case 1.—This 22-month-old black male infant was born prematurely and intubated for 1 month in the neonatal period for res¬ piratory distress syndrome. His history was significant for transient reactive air¬ way disease and pneumonia. The child pre¬
sented with cough, stridor, and increasing distress at 8 months of age at which time microlaryngoscopy and bronchoscopy revealed a 75% subglottic com¬ promise from cystic lesions in the posterior and right lateral subglottis. These cysts were subsequently removed endoscopically, and follow-up with microlaryngoscopy and bronchoscopy 1 month later revealed a nor¬ mal subglottis. This child has had no stridor or upper airway compromise since. Case 2.—This 3 Vè-year-old girl was born prematurely and intubated at birth for 4 hours for suspected respiratory distress syndrome, which was later not confirmed, and the child was extubated. On the 14th day of life she was reintubated for a period of 4 days because of increasingly prolonged apnea spells. Following this, she was treated medically for necrotizing entero¬ colitis, which prolonged her hospitalization to a total of 2 months from the time of birth. Shortly after being discharged the child developed stridor and increasing respira¬ tory distress, for which she underwent bronchoscopy and was found to have severe
respiratory
subglottic stenosis; a tracheotomy was per¬ formed. Subsequently, her airway was well maintained with a tracheotomy, and she was referred from Minneapolis, Minn, to Cincinnati, where laryngoscopy and bron¬ choscopy showed an irregular mucosal-covered soft subglottic stenosis. A laryngotracheoplasty was then performed with ante¬ rior costal cartilage graft. At the time of surgery submucosal
cysts were found to be
present in addition
to the stenosis, and these were removed. The stenosis was found to be mainly left lateral but was also poste¬ rior and right lateral. The child had an un¬ complicated postoperative course, with early removal of the stent and early decan-
nulation. On follow-up with microlaryngoscopy and bronchoscopy she was found to have a good result with an adequate subglottic lu¬ men and a viable anterior graft in position.
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Case 3.—This 16-month-old white was a
boy
premature infant who required
a
ventilator for the first 10 days of life for respiratory distress syndrome. He then re¬ quired supplemental oxygen for a period of 4 months and developed bronchopulmonary dysplasia requiring use of furosemide (Lasix), spironolactone (Aldactone), and theophylline (Theo-Dur). He then had an episode of suspected croup, with respira¬ tory distress and cyanosis requiring 3 days of intubation at 4 Vá months of age. Follow¬ ing this episode, he had continuing stridor and he underwent a microlaryngoscopy and bronchoscopy at 9 months of age that re¬ vealed a large left subglottic cyst. This cyst was removed with diathermy using an en¬ doscopie approach, and 1 week later the la¬ ser was used endoscopically for removal of the remaining small left posterior and an¬ terior cysts. Since that time the patient has done well and has not had stridor or recur¬ rent
cyanosis.
Case 4.—This 2-year-old white boy was born prematurely and intubated for 10 weeks in the neonatal period for respira¬ tory distress syndrome. His history was significant for patent ductus arteriosus li¬ gation. The child presented at 20 months of age with frequent episodes of respiratory distress and two episodes of cyanosis. Mi¬ crolaryngoscopy and bronchoscopy re¬ vealed subglottic cysts on the anterior and posterior walls. These were removed in three endoscopie procedures. Since then the patient has been well and has had no recur¬ rence of respiratory distress or cyanotic
spells. Case 5.—This male infant was born at 29 weeks' gestation and weighed 1170 g. He had immediate respiratory problems neces¬ sitating intubation for 5 days. There were two subsequent intubations for 2 and 6 days each. There were mild retractions and chronic bronchopulmonary dysplasia, but the child was discharged at 2 months of age. Two months after discharge the child was readmitted with bronchitis, tachypnea, wheezing, and retractions. There was bi¬ phasic stridor, but this improved and he was discharged 3 days later. The wheezing persisted and the child was readmitted at age 5 months with increasing biphasic stri¬ dor and retractions. Direct laryngoscopy and bronchoscopy revealed a circumferen-
Fig 1.—Subglottic area plasty with cyst in situ.
at
laryngotracheo-
Fig 2.—Removed subglottic cyst.
tial soft subglottic stenosis that was easily dilated with a 3-mm bronchoscope. At age 6 months the biphasic stridor had returned and the patient underwent re¬ peated bronchoscopy and dilatation with a 3.5-mm bronchoscope. At age 8 months, symptoms recurred and again bronchos¬ copy revealed the soft stenosis that was di¬ lated with a 3.5-mm bronchoscope, but the collapsing tissue necessitated a tracheot¬ omy. The child continued to have persistent
pulmonary problems. Repeated bronchoscopies
were
per¬
formed at 6-month intervals without im¬ provement of the stenosis. At age 26
months, an Evans-type castellating laryngotracheoplasty with an Aboulker stent was performed. At the time of surgery a bronchoscopy revealed three mounded ar¬ eas in the subglottic area. On opening the subglottic area the cricoid appeared nor¬ mal, but deep below the mucosa were three small inclusion cysts containing clear fluid (Figs 1 through 3). The stent was left in place for 2 months, and 3 months later the patient was successfully decannulated. One year later he had had no further upper air¬ way problems or stridor.
Case 6.—This male infant
was
27 weeks
gestational age, weighed 950 g, and was in¬ tubated shortly after birth with a 2.5-mm endotracheal tube. At 8 days of age a patent
Fig 3.—Photomicrograph of subglottic cyst with cuboidal epithelial lining and surrounding fibrous (magnification X100).
tissue
ductus arteriosus was ligated. He developed Candida colonization of the lungs, but was extubated at 20 days of age. He was hospi¬ talized for a total of 75 days with long-term carbon dioxide retention and bronchopulmonary
dysplasia.
discharge he had in¬ creasing respiratory distress associated with an upper respiratory tract infection. He was admitted, improved rapidly, and discharged 6 days later. Four days after discharge he was read¬ mitted with inspiratory stridor. On flexible nasopharyngoscopy a subglottic mass was noted. At bronchoscopy three subglottic cysts were identified and marsupialized One month after
with the carbon dioxide laser. There was no recurrence after 6 months. Case 7.—This infant was born at 29 weeks' gestational age, weighed 950 g, and was intubated 2 hours after delivery, and he remained intubated for 3 weeks. After extubation the child had chronic problems with carbon dioxide retention, tachypnea, and required 40% oxygen. At age 3 months
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discharge was imminent, but the child be¬ "funny breathing" with feed¬ ings. Pulmonary consultation was obtained and the patient was noted to have inspiratory and expiratory stridor. Flexible nasopharyngoscopy was performed and a sub¬ glottic mass was noted. At bronchoscopy there was a thickened mass on the left lateral subglottic wall that was removed with the laser. The patient was intubated for 24 hours, then was extubated, and did well. Three years later there gan to have
was no
evidence of
recurrence.
Case 8.—This premature female infant was born at 27 weeks' gestation and weighed 900 g. She was intubated for 5 days
for respiratory distress syndrome, but was then extubated and initially did well. She had progressive symptoms of stridor at about 8 months of age and, on endoscopy, was noted to have subglottic cysts. These were removed with diathermy at two sepa¬ rate procedures 1 week apart. She had no evidence of recurrence after 2 years. Case 9.—This premature female infant
born at 30 weeks' gestation and weighed 1000 g. She was intubated for 90 days, but had multiple complications in¬ cluding severe bronchopulmonary dyspla¬ sia. At 1 year of age a diagnosis of severe subglottic stenosis and subglottic cysts was made. She had multiple endoscopie pro¬ cedures, but because of the severity of the subglottic stenosis a tracheotomy was performed. A laryngotracheoplasty was planned but she died of bronchopulmonary dysplasia complicated by a respiratory syncytial virus infection.
TREATMENT
was
removed, follow-up with endoscopy would be repeated in 4 to 6 weeks.
All patients with biphasic stridor and/or breathing difficulties need examination of the airway. An initial examination may be performed with flexible nasopharyngoscopy as described by Fan.5 If no pathologic conditions are found to explain the symp¬ toms, or if the examination is suboptimal, examination of the entire laryngotracheobronchial tree should be performed with the patient under general anesthesia using fiberoptic telescopic equipment. If at this examination there are pathologic findings in the subglottic area, a distinction between a subglottic stenosis and subglottic cysts should be made. A subglottic stenosis will usually be circumferential, is firm, and may not be amenable to bronchoscopic dilata¬ tion. Subglottic cysts will either be obvious or
present with intact mucosa, with
COMMENT
Over the past 5 years
one or
soft mounds coming from the subglot¬ tic walls immediately below the vocal cords (Fig 4). Usually the bronchoscope may be passed through the obstruction with rela¬ tive ease. If the cyst is thin walled it may actually rupture with this maneuver and provide the definitive treatment. If not, the cyst may be marsupialized using a cup for¬ ceps, endoscopie diathermy, or the carbon dioxide laser if it is available. We would give intraoperative steroids and observe the patient in the pediatrie intensive care unit postoperatively. If the patient does well and it is believed that all the cysts were
Case Report Gestational Case
Age,
wk
Birth
Weight,
g 1400
Intubation Length, d
Times Intubated
have inde¬
eral weeks to months later. In most cases the diagnosis of subglottic cysts was apparent at endoscopy, but in two patients a diagnosis of subglottic stenosis was made, a tracheotomy was done for an airway, and the cysts were recognized at the time of laryngotracheoplasty. In both of these patients the cysts were deep in the submucosa and appeared as a soft collapsing sub¬ glottic stenosis. The borders were rounded with intact mucosa rather than a tight circumferential stenosis. In retrospect, a patient born prema¬ turely with a history of intubation and delayed onset of subglottic stenosis should be carefully examined for pos¬ sible subglottic cysts. In those patients with obvious subglottic cysts, marsu¬ pialization with a cup forceps, diather¬ my, or carbon dioxide laser provided satisfactory treatment without recur-
more
Fig 4.—Endoscopie view of subglottic cyst.
we
pendently recognized an entity of sub¬ glottic cysts in patients who were born prematurely, intubated for varying lengths of time, and then developed symptoms of subglottic stenosis sev¬
Summary* From Extubation to Diagnosis, mo
Treatment
Endoscopie
Outcome
removal
No
recurrence over 14
mo
26
4, 4
1%
Tracheotomy for sub¬ glottic stenosis, 3-moold;
at age 3 y an LTP
was
Adequate
lumen
after
LTP and anterior
car¬
tilage graft
performed with
the finding submucosal cysts 1090
10, 3
Endoscopie
diathermy
No recurrence
over
8
mo
No recurrence
over
6
mo
followed In 1 wk with laser removal of re¬
1170
5, 2, and
maining cysts Endoscopie removal in 3 procedures Tracheotomy, 7-mo-old; LTP at 26
950 27
950
carbon di¬ oxide laser removal
Endoscopie 20
Endoscopie carbon oxide laser
No recurrence 1 y
mo
di¬
No
recurrence
2Vi y
No recurrence 1 y
marsupial¬
ization
Endoscopie diathermy with 2 procedures 1
No recurrence after 2 y
wk apart Several
endoscopie pro¬
cedures, severe sub¬ glottic stenosis, tra¬
cheotomy
Expired
from BPD
planned BPD indicates
bronchopulmonary dysplasia; LTP, laryngotracheoplasty.
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com¬
plicated by a respira¬ tory syncytial virus in¬ fection, LTP was
in six of our cases, although some more than one procedure to remove all the cysts (Table). The two patients who had a laryngotracheoplasty also had satisfactory airways without recurrence of the cysts. One rence
required
patient died of lung complications not associated with the subglottic cysts.
Subglottic lesions usually present history of biphasic stridor, cyspells, recurrent apnea, and feeding problems. They may be due to congenital lesions such as subglottic hemangiomas or lymphangiomas, con¬ genital subglottic stenosis, and postin¬ tubation subglottic stenosis.4·7 In 1968 Wigger and Tang6 reported a with a anotic
premature infant intubated for 5 weeks who developed multiple compli¬ cations and died at 3% months of age. At autopsy a 5-mm subglottic mucus retention cyst was found to occlude 80% of the lumen. On microscopic ex¬ amination there was intact mucosa, but surrounding scar tissue and mi¬ croscopic mucus retention cysts were noted. There were no further reports of similar findings until 1981 when Couriel and Phelan2 presented three pre¬ mature infants, all weighing less than 1000 g at birth, who had been intubated and who had delayed onset of subglot¬ tic narrowing diagnosed as subglottic cysts on direct laryngoscopy.2 They made the point that subglottic narrow¬ ing in the immediate extubation period is secondary to mucosal edema and that delayed obstruction weeks to months later is usually due to scarring and subglottic stenosis. In two pa¬ tients the subglottic cysts were rela¬ tively thin and easily ruptured with a bronchoscope without recurrence, but one had to be treated a second time with the carbon dioxide laser and a short intubation period. Fan,5 in presenting a technique for fiberoptic endoscopy in children, diag¬ nosed a subglottic cyst in a 14-monthold infant who had been intubated in the neonatal period. This was removed surgically and no recurrence was re¬
ported. Holinger et al8 have presented 26 patients who had an anterior cricoid split for either primary or secondary subglottic stenosis. Three of these pa¬ tients had subglottic cysts. One patient
had a tracheotomy after failure of the anterior cricoid split. The stenosis was recognized, but the diagnosis of sub¬ glottic cysts was not made until the patient died of other causes and a postmortem examination was per¬ formed. There were multiple cysts and hyperplasia of the subglottic mucus glands on microscopic examination. There are three other reports of subglottic cysts. Dagan et al3 described
2-month-old infant who presented with stridor and respiratory distress; she was intubated for 2 days, and then a
direct laryngoscopy was found to have a subglottic mucocele. Cham¬ berlain1 reported a newborn with a subglottic cyst diagnosed shortly after birth. The patient had a tracheotomy, but died at 3 months of age. At autopsy there was a 1-cm cyst below the cords that was lined with pseudostratified ciliated columnar epithelium, but was covered with normal mucosa without inflammation or scarring, although there were some microscopic daughter cysts in the area. The third case was a 17-year-old girl presented by DeSanto et al,4 in a review of 238 cysts of the larynx over a 20-year period. This was a bilobed cyst with the largest compo¬ nent (3 cm in diameter) externally and a 1-cm cyst internally in the subglottic area. It seemed to be similar to a flu¬ id-filled laryngocele, but the compo¬ nents communicated directly through the thyroid cartilage that was believed to be in the area of a vascular channel. From this review it would appear that there are rare congenital subglot¬ tic cysts,1-3-4 but that there is a definite emerging entity of subglottic mucus retention cysts as a complication of intubation in premature infants.2 We believe that the endotracheal tube pro¬ duces erosion, inflammation, and then scarring in the subglottic area that re¬ sults in sealing off the ducts of submucus glands and results in cyst forma¬ tion. In several of our cases, as well as those reported in the literature, the cysts were multiple and there was mi¬ croscopic evidence of scarring in the immediate area.6 With the expanding role of neonatology units and the increased survival rate in premature infants, this com¬ plication will no doubt be more fre¬ quently recognized. As otolaryngologists we are aware of the immediate on
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problems from long-term intubation, such as subglottic mucosal edema, ero¬ sions, and granulations that can make extubation difficult.9 Cotton and Seid,10 as well as others, have used the ante¬ rior cricoid split procedure to prevent the need for tracheotomies in a high percentage of these patients,811 The most commonly recognized delayed complication of long-term intubations
has been cicatricial circumferential subglottic stenosis, which if sympto¬ matic requires a tracheotomy and is usually treated definitively by some type of laryngotracheoplasty.12 We must also be aware of the possibility that subglottic cysts may also be a de¬ layed complication of intubation in the premature infant. Although there have been isolated reports on this condition, it has not been widely recognized by otolaryngologists and neonatologists. We call at¬ tention to this entity so that it may be more widely recognized and treated appropriately with marsupialization of the cysts rather than being treated as a subglottic stenosis with tracheo-
stomy. References 1. Chamberlain D. Congenital subglottic cyst of the larynx: a case report. Laryngoscope. 1970; 80:254-259. 2. Couriel JM, Phelan PD. Subglottic cysts: a complication of neonatal endotracheal intubation? Pediatrics. 1981;68:103-105. 3. Dagan R, Leiberman A, Strauss R, Bar-Zi J, Hirsch M. Subglottic mucocele in an infant. Pediatr Radiol. 1979;8:119-121. 4. DeSanto LW, Devine KD, Weiland LH. Cysts of the larynx: classification. Laryngoscope.
1970;80:145-176.
5. Fan LL. Transnasal fiberoptic endoscopy in children with obstructive apnea. Crit Care Med.
1984;12:590-592. 6. Wigger HJ, Tang M, Fatal laryngeal obstruction by iatrogenic cyst. J Pediatr. 1968;72:815-820. 7. Holinger RH, Brown WT. Congenital webs, cysts, laryngoceles and other anomalies of the larynx. Ann Otol Rhinol Laryngol. 1967;76:744. 8. Holinger LD, Stankiewicz JA, Livingston GL. Anterior cricoid split: the Chicago experience with an alternative to tracheotomy. Laryngoscope. 1987;97:19-24. 9. Hawkins DB. Hyaline membrane disease of the neonate prolonged intubation in management: effects on the larynx. Laryngoscope. 1978;88:201-224. 10. Cotton RT, Seid AB. Management of the extubation problem in the premature child: anterior cricoid split as an alternative to tracheotomy. Ann Otol Rhinol Laryngol. 1980;89:508-511. 11. Miller RH, Weatherly RA. Experience with anterior cricoid split for difficult neonatal extubation. Arch Otolaryngol Head Neck Surg. 1986;112:972-975. 12. Dankle SK, Schuller DE, McClead RE. Risk factors for neonatal acquired subglottic stenosis. Ann Otol Rhinol Laryngol. 1986;95:626-630.
Premature Infant
Smith, MD; Robin Cotton, MD; Charles M. Meyer III, MD
\s=b\ Supraglottic
cysts in the newborn
are
well-recognized entity, but subglottic cysts have been rarely reported. Over the past 6 years we have observed subglottic cysts in nine patients with relatively long a
intubations from the neonatal intensive care unit of two university hospitals. Most frequently the patients were extubated and did well for weeks or months, but then they had progressive biphasic stridor. On endoscopy the patients had a subglottic stenosis that was irregular but with a smooth mucosal lining. Usually the cysts were apparent, but in two patients the mucosa was thickened and the patients were treated as a subglottic stenosis with tracheostomy. These subglottic cysts were recognized at the time of laryngotracheoplasty. In six patients the cysts were managed either by marsupialization with cup forceps, endoscopic diathermy, or carbon dioxide laser without recurrence. It is our belief that this condition is most likely due to scarring and obstruction of mucus glands of the subglottic area from prolonged intubation. This entity should be recognized and looked for in the neonate who has an acquired subglottic stenosis and should first be treated conservatively with endoscopic marsupialization. (Arch Otolaryngol Head Neck Surg.
1990;116:479-482)
Although supraglottic cysts
and
laryngoceles are frequently rec¬ ognized, subglottic cysts have been rarely reported.16·8 Over the past 6 -ti-
years, in two separate neonatal inten¬
sive care units, we have had nine cases of subglottic mucus retention cysts in premature infants who had been intubated for varying lengths of time. REPORT OF CASES The following cases of subglottic cysts have been diagnosed at the Children's Hos¬ pital Medical Center, Cincinnati, Ohio, and
Accepted for publication September 19, 1989. From the Department of Otolaryngology\p=n-\Head and Neck Surgery, Oregon Health Sciences University, Portland (Dr Smith); and Department of Otolaryngology\p=n-\Maxillofacial Surgery, University of Cincinnati, Ohio (Drs Cotton and Meyer). Reprints not available.
Oregon Health Sciences University, Portland, from 1982 to 1988.
the
Case 1.—This 22-month-old black male infant was born prematurely and intubated for 1 month in the neonatal period for res¬ piratory distress syndrome. His history was significant for transient reactive air¬ way disease and pneumonia. The child pre¬
sented with cough, stridor, and increasing distress at 8 months of age at which time microlaryngoscopy and bronchoscopy revealed a 75% subglottic com¬ promise from cystic lesions in the posterior and right lateral subglottis. These cysts were subsequently removed endoscopically, and follow-up with microlaryngoscopy and bronchoscopy 1 month later revealed a nor¬ mal subglottis. This child has had no stridor or upper airway compromise since. Case 2.—This 3 Vè-year-old girl was born prematurely and intubated at birth for 4 hours for suspected respiratory distress syndrome, which was later not confirmed, and the child was extubated. On the 14th day of life she was reintubated for a period of 4 days because of increasingly prolonged apnea spells. Following this, she was treated medically for necrotizing entero¬ colitis, which prolonged her hospitalization to a total of 2 months from the time of birth. Shortly after being discharged the child developed stridor and increasing respira¬ tory distress, for which she underwent bronchoscopy and was found to have severe
respiratory
subglottic stenosis; a tracheotomy was per¬ formed. Subsequently, her airway was well maintained with a tracheotomy, and she was referred from Minneapolis, Minn, to Cincinnati, where laryngoscopy and bron¬ choscopy showed an irregular mucosal-covered soft subglottic stenosis. A laryngotracheoplasty was then performed with ante¬ rior costal cartilage graft. At the time of surgery submucosal
cysts were found to be
present in addition
to the stenosis, and these were removed. The stenosis was found to be mainly left lateral but was also poste¬ rior and right lateral. The child had an un¬ complicated postoperative course, with early removal of the stent and early decan-
nulation. On follow-up with microlaryngoscopy and bronchoscopy she was found to have a good result with an adequate subglottic lu¬ men and a viable anterior graft in position.
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Case 3.—This 16-month-old white was a
boy
premature infant who required
a
ventilator for the first 10 days of life for respiratory distress syndrome. He then re¬ quired supplemental oxygen for a period of 4 months and developed bronchopulmonary dysplasia requiring use of furosemide (Lasix), spironolactone (Aldactone), and theophylline (Theo-Dur). He then had an episode of suspected croup, with respira¬ tory distress and cyanosis requiring 3 days of intubation at 4 Vá months of age. Follow¬ ing this episode, he had continuing stridor and he underwent a microlaryngoscopy and bronchoscopy at 9 months of age that re¬ vealed a large left subglottic cyst. This cyst was removed with diathermy using an en¬ doscopie approach, and 1 week later the la¬ ser was used endoscopically for removal of the remaining small left posterior and an¬ terior cysts. Since that time the patient has done well and has not had stridor or recur¬ rent
cyanosis.
Case 4.—This 2-year-old white boy was born prematurely and intubated for 10 weeks in the neonatal period for respira¬ tory distress syndrome. His history was significant for patent ductus arteriosus li¬ gation. The child presented at 20 months of age with frequent episodes of respiratory distress and two episodes of cyanosis. Mi¬ crolaryngoscopy and bronchoscopy re¬ vealed subglottic cysts on the anterior and posterior walls. These were removed in three endoscopie procedures. Since then the patient has been well and has had no recur¬ rence of respiratory distress or cyanotic
spells. Case 5.—This male infant was born at 29 weeks' gestation and weighed 1170 g. He had immediate respiratory problems neces¬ sitating intubation for 5 days. There were two subsequent intubations for 2 and 6 days each. There were mild retractions and chronic bronchopulmonary dysplasia, but the child was discharged at 2 months of age. Two months after discharge the child was readmitted with bronchitis, tachypnea, wheezing, and retractions. There was bi¬ phasic stridor, but this improved and he was discharged 3 days later. The wheezing persisted and the child was readmitted at age 5 months with increasing biphasic stri¬ dor and retractions. Direct laryngoscopy and bronchoscopy revealed a circumferen-
Fig 1.—Subglottic area plasty with cyst in situ.
at
laryngotracheo-
Fig 2.—Removed subglottic cyst.
tial soft subglottic stenosis that was easily dilated with a 3-mm bronchoscope. At age 6 months the biphasic stridor had returned and the patient underwent re¬ peated bronchoscopy and dilatation with a 3.5-mm bronchoscope. At age 8 months, symptoms recurred and again bronchos¬ copy revealed the soft stenosis that was di¬ lated with a 3.5-mm bronchoscope, but the collapsing tissue necessitated a tracheot¬ omy. The child continued to have persistent
pulmonary problems. Repeated bronchoscopies
were
per¬
formed at 6-month intervals without im¬ provement of the stenosis. At age 26
months, an Evans-type castellating laryngotracheoplasty with an Aboulker stent was performed. At the time of surgery a bronchoscopy revealed three mounded ar¬ eas in the subglottic area. On opening the subglottic area the cricoid appeared nor¬ mal, but deep below the mucosa were three small inclusion cysts containing clear fluid (Figs 1 through 3). The stent was left in place for 2 months, and 3 months later the patient was successfully decannulated. One year later he had had no further upper air¬ way problems or stridor.
Case 6.—This male infant
was
27 weeks
gestational age, weighed 950 g, and was in¬ tubated shortly after birth with a 2.5-mm endotracheal tube. At 8 days of age a patent
Fig 3.—Photomicrograph of subglottic cyst with cuboidal epithelial lining and surrounding fibrous (magnification X100).
tissue
ductus arteriosus was ligated. He developed Candida colonization of the lungs, but was extubated at 20 days of age. He was hospi¬ talized for a total of 75 days with long-term carbon dioxide retention and bronchopulmonary
dysplasia.
discharge he had in¬ creasing respiratory distress associated with an upper respiratory tract infection. He was admitted, improved rapidly, and discharged 6 days later. Four days after discharge he was read¬ mitted with inspiratory stridor. On flexible nasopharyngoscopy a subglottic mass was noted. At bronchoscopy three subglottic cysts were identified and marsupialized One month after
with the carbon dioxide laser. There was no recurrence after 6 months. Case 7.—This infant was born at 29 weeks' gestational age, weighed 950 g, and was intubated 2 hours after delivery, and he remained intubated for 3 weeks. After extubation the child had chronic problems with carbon dioxide retention, tachypnea, and required 40% oxygen. At age 3 months
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discharge was imminent, but the child be¬ "funny breathing" with feed¬ ings. Pulmonary consultation was obtained and the patient was noted to have inspiratory and expiratory stridor. Flexible nasopharyngoscopy was performed and a sub¬ glottic mass was noted. At bronchoscopy there was a thickened mass on the left lateral subglottic wall that was removed with the laser. The patient was intubated for 24 hours, then was extubated, and did well. Three years later there gan to have
was no
evidence of
recurrence.
Case 8.—This premature female infant was born at 27 weeks' gestation and weighed 900 g. She was intubated for 5 days
for respiratory distress syndrome, but was then extubated and initially did well. She had progressive symptoms of stridor at about 8 months of age and, on endoscopy, was noted to have subglottic cysts. These were removed with diathermy at two sepa¬ rate procedures 1 week apart. She had no evidence of recurrence after 2 years. Case 9.—This premature female infant
born at 30 weeks' gestation and weighed 1000 g. She was intubated for 90 days, but had multiple complications in¬ cluding severe bronchopulmonary dyspla¬ sia. At 1 year of age a diagnosis of severe subglottic stenosis and subglottic cysts was made. She had multiple endoscopie pro¬ cedures, but because of the severity of the subglottic stenosis a tracheotomy was performed. A laryngotracheoplasty was planned but she died of bronchopulmonary dysplasia complicated by a respiratory syncytial virus infection.
TREATMENT
was
removed, follow-up with endoscopy would be repeated in 4 to 6 weeks.
All patients with biphasic stridor and/or breathing difficulties need examination of the airway. An initial examination may be performed with flexible nasopharyngoscopy as described by Fan.5 If no pathologic conditions are found to explain the symp¬ toms, or if the examination is suboptimal, examination of the entire laryngotracheobronchial tree should be performed with the patient under general anesthesia using fiberoptic telescopic equipment. If at this examination there are pathologic findings in the subglottic area, a distinction between a subglottic stenosis and subglottic cysts should be made. A subglottic stenosis will usually be circumferential, is firm, and may not be amenable to bronchoscopic dilata¬ tion. Subglottic cysts will either be obvious or
present with intact mucosa, with
COMMENT
Over the past 5 years
one or
soft mounds coming from the subglot¬ tic walls immediately below the vocal cords (Fig 4). Usually the bronchoscope may be passed through the obstruction with rela¬ tive ease. If the cyst is thin walled it may actually rupture with this maneuver and provide the definitive treatment. If not, the cyst may be marsupialized using a cup for¬ ceps, endoscopie diathermy, or the carbon dioxide laser if it is available. We would give intraoperative steroids and observe the patient in the pediatrie intensive care unit postoperatively. If the patient does well and it is believed that all the cysts were
Case Report Gestational Case
Age,
wk
Birth
Weight,
g 1400
Intubation Length, d
Times Intubated
have inde¬
eral weeks to months later. In most cases the diagnosis of subglottic cysts was apparent at endoscopy, but in two patients a diagnosis of subglottic stenosis was made, a tracheotomy was done for an airway, and the cysts were recognized at the time of laryngotracheoplasty. In both of these patients the cysts were deep in the submucosa and appeared as a soft collapsing sub¬ glottic stenosis. The borders were rounded with intact mucosa rather than a tight circumferential stenosis. In retrospect, a patient born prema¬ turely with a history of intubation and delayed onset of subglottic stenosis should be carefully examined for pos¬ sible subglottic cysts. In those patients with obvious subglottic cysts, marsu¬ pialization with a cup forceps, diather¬ my, or carbon dioxide laser provided satisfactory treatment without recur-
more
Fig 4.—Endoscopie view of subglottic cyst.
we
pendently recognized an entity of sub¬ glottic cysts in patients who were born prematurely, intubated for varying lengths of time, and then developed symptoms of subglottic stenosis sev¬
Summary* From Extubation to Diagnosis, mo
Treatment
Endoscopie
Outcome
removal
No
recurrence over 14
mo
26
4, 4
1%
Tracheotomy for sub¬ glottic stenosis, 3-moold;
at age 3 y an LTP
was
Adequate
lumen
after
LTP and anterior
car¬
tilage graft
performed with
the finding submucosal cysts 1090
10, 3
Endoscopie
diathermy
No recurrence
over
8
mo
No recurrence
over
6
mo
followed In 1 wk with laser removal of re¬
1170
5, 2, and
maining cysts Endoscopie removal in 3 procedures Tracheotomy, 7-mo-old; LTP at 26
950 27
950
carbon di¬ oxide laser removal
Endoscopie 20
Endoscopie carbon oxide laser
No recurrence 1 y
mo
di¬
No
recurrence
2Vi y
No recurrence 1 y
marsupial¬
ization
Endoscopie diathermy with 2 procedures 1
No recurrence after 2 y
wk apart Several
endoscopie pro¬
cedures, severe sub¬ glottic stenosis, tra¬
cheotomy
Expired
from BPD
planned BPD indicates
bronchopulmonary dysplasia; LTP, laryngotracheoplasty.
Downloaded From: http://archotol.jamanetwork.com/ by a Oakland University User on 06/08/2015
com¬
plicated by a respira¬ tory syncytial virus in¬ fection, LTP was
in six of our cases, although some more than one procedure to remove all the cysts (Table). The two patients who had a laryngotracheoplasty also had satisfactory airways without recurrence of the cysts. One rence
required
patient died of lung complications not associated with the subglottic cysts.
Subglottic lesions usually present history of biphasic stridor, cyspells, recurrent apnea, and feeding problems. They may be due to congenital lesions such as subglottic hemangiomas or lymphangiomas, con¬ genital subglottic stenosis, and postin¬ tubation subglottic stenosis.4·7 In 1968 Wigger and Tang6 reported a with a anotic
premature infant intubated for 5 weeks who developed multiple compli¬ cations and died at 3% months of age. At autopsy a 5-mm subglottic mucus retention cyst was found to occlude 80% of the lumen. On microscopic ex¬ amination there was intact mucosa, but surrounding scar tissue and mi¬ croscopic mucus retention cysts were noted. There were no further reports of similar findings until 1981 when Couriel and Phelan2 presented three pre¬ mature infants, all weighing less than 1000 g at birth, who had been intubated and who had delayed onset of subglot¬ tic narrowing diagnosed as subglottic cysts on direct laryngoscopy.2 They made the point that subglottic narrow¬ ing in the immediate extubation period is secondary to mucosal edema and that delayed obstruction weeks to months later is usually due to scarring and subglottic stenosis. In two pa¬ tients the subglottic cysts were rela¬ tively thin and easily ruptured with a bronchoscope without recurrence, but one had to be treated a second time with the carbon dioxide laser and a short intubation period. Fan,5 in presenting a technique for fiberoptic endoscopy in children, diag¬ nosed a subglottic cyst in a 14-monthold infant who had been intubated in the neonatal period. This was removed surgically and no recurrence was re¬
ported. Holinger et al8 have presented 26 patients who had an anterior cricoid split for either primary or secondary subglottic stenosis. Three of these pa¬ tients had subglottic cysts. One patient
had a tracheotomy after failure of the anterior cricoid split. The stenosis was recognized, but the diagnosis of sub¬ glottic cysts was not made until the patient died of other causes and a postmortem examination was per¬ formed. There were multiple cysts and hyperplasia of the subglottic mucus glands on microscopic examination. There are three other reports of subglottic cysts. Dagan et al3 described
2-month-old infant who presented with stridor and respiratory distress; she was intubated for 2 days, and then a
direct laryngoscopy was found to have a subglottic mucocele. Cham¬ berlain1 reported a newborn with a subglottic cyst diagnosed shortly after birth. The patient had a tracheotomy, but died at 3 months of age. At autopsy there was a 1-cm cyst below the cords that was lined with pseudostratified ciliated columnar epithelium, but was covered with normal mucosa without inflammation or scarring, although there were some microscopic daughter cysts in the area. The third case was a 17-year-old girl presented by DeSanto et al,4 in a review of 238 cysts of the larynx over a 20-year period. This was a bilobed cyst with the largest compo¬ nent (3 cm in diameter) externally and a 1-cm cyst internally in the subglottic area. It seemed to be similar to a flu¬ id-filled laryngocele, but the compo¬ nents communicated directly through the thyroid cartilage that was believed to be in the area of a vascular channel. From this review it would appear that there are rare congenital subglot¬ tic cysts,1-3-4 but that there is a definite emerging entity of subglottic mucus retention cysts as a complication of intubation in premature infants.2 We believe that the endotracheal tube pro¬ duces erosion, inflammation, and then scarring in the subglottic area that re¬ sults in sealing off the ducts of submucus glands and results in cyst forma¬ tion. In several of our cases, as well as those reported in the literature, the cysts were multiple and there was mi¬ croscopic evidence of scarring in the immediate area.6 With the expanding role of neonatology units and the increased survival rate in premature infants, this com¬ plication will no doubt be more fre¬ quently recognized. As otolaryngologists we are aware of the immediate on
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problems from long-term intubation, such as subglottic mucosal edema, ero¬ sions, and granulations that can make extubation difficult.9 Cotton and Seid,10 as well as others, have used the ante¬ rior cricoid split procedure to prevent the need for tracheotomies in a high percentage of these patients,811 The most commonly recognized delayed complication of long-term intubations
has been cicatricial circumferential subglottic stenosis, which if sympto¬ matic requires a tracheotomy and is usually treated definitively by some type of laryngotracheoplasty.12 We must also be aware of the possibility that subglottic cysts may also be a de¬ layed complication of intubation in the premature infant. Although there have been isolated reports on this condition, it has not been widely recognized by otolaryngologists and neonatologists. We call at¬ tention to this entity so that it may be more widely recognized and treated appropriately with marsupialization of the cysts rather than being treated as a subglottic stenosis with tracheo-
stomy. References 1. Chamberlain D. Congenital subglottic cyst of the larynx: a case report. Laryngoscope. 1970; 80:254-259. 2. Couriel JM, Phelan PD. Subglottic cysts: a complication of neonatal endotracheal intubation? Pediatrics. 1981;68:103-105. 3. Dagan R, Leiberman A, Strauss R, Bar-Zi J, Hirsch M. Subglottic mucocele in an infant. Pediatr Radiol. 1979;8:119-121. 4. DeSanto LW, Devine KD, Weiland LH. Cysts of the larynx: classification. Laryngoscope.
1970;80:145-176.
5. Fan LL. Transnasal fiberoptic endoscopy in children with obstructive apnea. Crit Care Med.
1984;12:590-592. 6. Wigger HJ, Tang M, Fatal laryngeal obstruction by iatrogenic cyst. J Pediatr. 1968;72:815-820. 7. Holinger RH, Brown WT. Congenital webs, cysts, laryngoceles and other anomalies of the larynx. Ann Otol Rhinol Laryngol. 1967;76:744. 8. Holinger LD, Stankiewicz JA, Livingston GL. Anterior cricoid split: the Chicago experience with an alternative to tracheotomy. Laryngoscope. 1987;97:19-24. 9. Hawkins DB. Hyaline membrane disease of the neonate prolonged intubation in management: effects on the larynx. Laryngoscope. 1978;88:201-224. 10. Cotton RT, Seid AB. Management of the extubation problem in the premature child: anterior cricoid split as an alternative to tracheotomy. Ann Otol Rhinol Laryngol. 1980;89:508-511. 11. Miller RH, Weatherly RA. Experience with anterior cricoid split for difficult neonatal extubation. Arch Otolaryngol Head Neck Surg. 1986;112:972-975. 12. Dankle SK, Schuller DE, McClead RE. Risk factors for neonatal acquired subglottic stenosis. Ann Otol Rhinol Laryngol. 1986;95:626-630.
