Upper Airway Obstruction From Foregut Cysts of the Hypopharynx By Timothy G. Conty and W. Hardy
Hendren
T
HIS REPORT describes experiences with upper airway obstruction in three neonates caused by cysts of foregut origin within the hypopharynx. Foregut cysts, including esophageal duplications and bronchogenic cysts, have been reported in this location extremely rarely but have not been associated with airway obstruction. Is2 Each of these cases required emergency establishment of an airway before dealing with the lesion. CASE
REPORTS
Case I C.B. (MGH No. 154-l&12), an 8 lb, 4 oz female infant had severe respiratory distress in the delivery room immediately after birth. Direct laryngoscopy revealed a large smooth-walled, cystic mass occupying the hypopharynx, completely obstructing the airway. With difficulty, a small endotracheal tube was passed, and the patient was referred. Tracheostomy was performed. Using an adult laryngoscope for exposure, a large cystic mass arising from the left posterior pharyngeal wall was unroofed (Fig. IA). The cyst was thick-walled and contained thick mucous.
Postoperatively, the patient required respiratory support for 5 days. The tracheostomy tube was removed at 26 days of age, and the patient was discharged at 30 days. On histophathologic examination, the cyst was composed of an external layer of squamous epithelium, a thick-middle musculotibrous layer and an inner lining of respiratory epithelium with subepithelial mucous glands (Fig. 1B). The pathologic diagnosis was bronchogenic cyst.
Case 2 C.L. (MGH No. 166-29-89), a IO-wk-old female infant of difficulty in swallowing and two episodes of severe well between episodes, except for continued spitting-up chusetts General Hospital, she was a vigorous infant in upper lobe pneumonia, confirmed by chest roentgenogram. gurgitation at the pharyngeal level of about half of each
was admitted because of recurrent bouts respiratory distress. The child appeared of feedings. On referral to the Massano distress but had clinical signs of right A tine barium swallow revealed reswallowed bolus, but that which passed
through the pharynx passed down to the esophagus normally. Palpation of the pharynx suggested a mass. Surgical consultation was sought on her tenth hospital day. On direct laryngoscopy, a cystic mass was observed at the base of the tongue. During this procedure, the child’s airway became completely obstructed, requiring emergency intubation. She was taken to the operating room immediately where tracheostomy was performed over a No. 3 bronchoscope. On repeat laryngoscopy in the operating room, a 3-cm mass arising from the fold between the base of the tongue and the epiglottis was unroofed (Fig. 2). The wall of the cyst was thick and contained thick mucoid material. Postoperatively, the child began taking feedings normally on the second day without respiratory
From the Division of Pediatric Surgery, Massachusetts General Hospital, and the Department of Surgery, Harvard Medical School, Boston, Mass. Presented before the 6th Annual Meeting of the American Pediatric Surgical Association. San Juan, Puerto Rico, April 10-12. 1975. Address for reprint requests: W. Hardy Hendren. M.D.. Massachusetts General Hospital, Boston. Mass. 02114. rQ1975 by Grune & Stratton. Inc. Journal of Pediatric Surgery, Vol. 10, No. 5 (October), 1975
807
CANTY
CASE
AND
HENDREN
I
Fig. 1. Case 1. (A) Diagrammatic representation of large hypopharyngeal lesion completely obstructing glottic inlet. (8) Photomicrograph showing an external mucosal lining of squamous epithelium, a thick middle layer of musculofibrous tissue, and an inner lining of respimtory epithelium. Note presence of subepithelial mucous glands.
Based
on tongue
/
Fig. 2. Caso 2. Diagmmmatic representation of cystic Ierion arising at base of tongue and obstructing glottic inlet.
FOREGUT
CYSTS
809
distress. Her tracheostomy tube was removed on the seventh postoperative day, and the child was discharged on the tenth day. Histologic examination of the cyst wall revealed both surfaces to be covered with squamous epithelium separated by a thick inner layer of skeletal muscle. This was consistent with an upper esophageal duplication.
Case 3 R.M. (MGH No. 177-03-88). a lo-day-old male infant was referred because of persistent upper airway obstruction and a mass in the posterior pharynx, encroaching on the larynx. Laryngoscopy under anesthesia revealed a large cystic mass arising in the left pyriform sinus, almost covering the glottic inlet (Fig. 3). Endotracheal intubation was performed. The cyst wall was unroofed with cautery, releasing thick, mucoid material. The child was extubated. breathed well, and tracheostomy was not needed, ReexaminaHe was well for 5 mo but then began to exhibit recurrence of respiratory distress. tion under anesthesia revealed recurrence of the cystic mass. It was unroofed again, relieving his symptoms. Symptoms recurred once again 1 mo later, again requiring laryngoscopy and intubation. On this occasion, the outer wall of the mass was extensively resected, unroofing it several centimeters downward to the level of the cricoid cartilage. The considerable extent of the lesion had not been appreciated at the first procedures. Histopathology of the cyst showed it to be thick-walled. lined with respiratory epithelium on one side and squamous epithelium on the other, separated by a thick, muscular layer. consistent with a bronchogenic foregut cyst,
Case 4 In contrast to these three cases of cysts of foregut origin is a fourth case of a simple laryngeal cyst that proved lethal to the infant. J. C. (MGH No. 120-77-35). a l-day-old male infant was referred because of upper airway obstruction. He was noted to be cyanotic at birth with crowing respirations. He was in severe respiratory distress on admission. Under anesthesia, laryngoscopy revealed a large cyst arising from the left laryngeal wall almost completely blocking the inlet, A bron-
Fig. 3.
Diagram of lesion in care 3.
810
CANM
AND HENDREN
Fig. 4. Case 4. (A) Photograph of larynx at autopsy showing cystic lesion arising in laryngeal inlet. (6) Photomicrograph of wall of simple cyst of larynx. Note thin wall composed of a few layen of simple stroma in contrast to thick muscular wall seen in Fig. 1 B.
choscope was then passed, and tracheostomy was performed using a silver metal tracheostomy tube. This event was prior to the era when present day plastic tubes were in use. The child did well for 5 days, then suddenly died. At postmortem examination, it was found that the tip of the metal tracheostomy tube had perforated the trachea. The large thin-walled cyst was noted within the left lateral wall of the larynx markedly narrowing the glottic inlet (Fig. 4A). Histopathologic examination of this showed a thin-walled cyst composed only of a single layer of lining cells and a thin fibrous capsule (Fig. 4B) This was considered to be a simple laryngeal cyst. DISCUSSION
Respiratory distress in.the newborn period secondary to apparent airway obstruction is most commonly due to congenital laryngeal stridor or laryngomalacia.3q4 This condition rarely requires surgical intervention and usually clears spontaneously in a few months. It is best recognized at direct laryngoscopy where one sees the floppy glottic folds that, on inspiration, are drawn in and partially occlude the airway. Other causes of airway obstruction include congenital webs and stenoses of the larynx; these often do not cause difficulties until later in infancy. Holinger reported congenital subglottic stenosis as the most common anomaly of the tracheobronchial tree requiring tracheostomy prior to 1 yr of age? Other lesions include lymphoma and hemangioma, which can impinge on the larynx from outside and cause respiratory distress. Cystic lesions of the larynx arising in the newborn are rare. The majority of these are laryngoceles arising from the ventricles of the larynx. These thinwalled, air-containing cysts may assume large size and cause airway obstruction in the newborn. Laryngoceles are best treated by immediate direct laryngoscopy and aspiration of the cyst. They may occasionally recur, requiring excision.“6
FOREGUT
CYSTS
811
Simple mucous cysts of the larynx, as typified by case 4, are also rare. Holinger reported only three cases in a 30-yr experience.4 De Santo et al. found 190 cases in the Mayo Clinic records of cysts of the larynx in all ages covering a 20-yr period.5 These cysts rarely caused life-threatening airway obstruction. Some believe these lesions to be simple retention cysts resulting from obstruction of mucous gland ducts. They may occur anywhere in the larynx from the aryepiglottic fold to the laryngeal ventricle and are best treated by transoral aspiration and partial excision of the cyst wall to prevent recurrence. When airway obstruction is severe, establishment of an adequate airway by emergency bronchoscopy and tracheostomy is necessary. Cysts of the type presented by cases 1, 2, and 3 are apparently quite rare. In 1940, Ladd and Gross described an 1 I-mo-old infant presenting with difficulty in swallowing secondary to a duplication cyst at the base of the tongue; this was excised without difficulty;’ it was a colonic duplication. Daudet, reporting in a symposium on gastrointestinal duplications in 1965, made mention of a personal communication regarding three infants with duplication cysts of the oropharynx.2 Respiratory distress, however, was not a presenting symptom in any of these cases. These thick-walled, multilayered, cystic lesions containing layers of muscle and organized epithelial coverings on both sides were classified by Ravitch as cysts of foregut origin.7 They are further divided into three categories: (I) Enteric cysts, which are lined by columnar or ciliated epithelum, frequently contain ectopic gastric mucosa, usually lie in the posterior mediastinum, and are a form of esophageal duplication. They are frequently associated with abnormalities of the vertebral bodies. (2) Bronchogenic cysts, which are lined by respiratory epithelium with a poorly developed muscle wall, commonly arise from the bronchi. (3) Esophageal cysts, often lying intramurally within the esophagus, are not associated with vertebral abnormalities. cases 1 and Our three cases are best classified as foregut cysts. Furthermore, 3 fit the bronchogenic cyst category, and case 2, the esophageal type. The histopathologic classification is of importance in regard to treatment. Simple aspiration of this type of cyst, as is done for laryngoceles and which was practiced for many years for mediastinal duplication cysts, is rarely successful.s Duplication cysts must be adequately unroofed or recurrence is common. This point is clearly underlined by our experience with case 3. It is puzzling to note that this report of foregut cysts in this location causing airway obstruction is unique in the literature. These lesions are most likely much more common than previously supposed. Careful histopathologic examination and classification of resected cystic lesions from this location will be necessary to reveal their true incidence. These four cases emphasize the importance of direct laryngoscopy and bronchoscopy for the immediate diagnosis and treatment of severe respiratory distress secondary to airway obstruction. Cystic lesions are rare causes of airway obstruction; however, the presenting symptoms of inspiratory stridor and sternal retraction should alert physicians to this possibility and prompt direct visualization of the oropharynx and larynx. Definitive treatment can then be readily carried out.
812
CANTY
AND HENDREN
REFERENCES I. Ladd WE, Gross RE: Surgical treatment of duplications of the alimentary tract. Surg Gynecol Obstet 70: 295-307, 1940 2. Daudet M: Symposium Consacre aux duplications intestinales. Ann Chir Infantile 8: 5-80, 1967 3. Shackelford GD, McAlister WH: Congenital laryngeal cyst. Am J Roentgenal 114: 289-292, 1972 4. Holinger PH. Brown WT: Congenital webs, cysts, laryngoceles and other anomalies of the larynx. Ann Otol Rhino1 Laryngol 76: 744-752, 1967
5. De Santo LW, Devine KD, Weiland LH: Cysts of the larynx-classification. Laryngoscope 80: 145-176, 1970 6. Suehs OW, Powel DB: Congenital cysts of the larynx in infants. Laryngoscope 77: 654-662, 1967 7. Ravitch MM, Sabiston DC: Mediastinal infections and tumors, in Mustard WT. Ravitch MM, Snyder WH Jr, et al (eds): Pediatric Surgery, Chicago, Year Book, 1969, pp 417-420 8. Bishop HC, Koop CE: Surgical management of duplications of the alimentary tract. Am J Surg 107: 434-442, 1964
Hendren
T
HIS REPORT describes experiences with upper airway obstruction in three neonates caused by cysts of foregut origin within the hypopharynx. Foregut cysts, including esophageal duplications and bronchogenic cysts, have been reported in this location extremely rarely but have not been associated with airway obstruction. Is2 Each of these cases required emergency establishment of an airway before dealing with the lesion. CASE
REPORTS
Case I C.B. (MGH No. 154-l&12), an 8 lb, 4 oz female infant had severe respiratory distress in the delivery room immediately after birth. Direct laryngoscopy revealed a large smooth-walled, cystic mass occupying the hypopharynx, completely obstructing the airway. With difficulty, a small endotracheal tube was passed, and the patient was referred. Tracheostomy was performed. Using an adult laryngoscope for exposure, a large cystic mass arising from the left posterior pharyngeal wall was unroofed (Fig. IA). The cyst was thick-walled and contained thick mucous.
Postoperatively, the patient required respiratory support for 5 days. The tracheostomy tube was removed at 26 days of age, and the patient was discharged at 30 days. On histophathologic examination, the cyst was composed of an external layer of squamous epithelium, a thick-middle musculotibrous layer and an inner lining of respiratory epithelium with subepithelial mucous glands (Fig. 1B). The pathologic diagnosis was bronchogenic cyst.
Case 2 C.L. (MGH No. 166-29-89), a IO-wk-old female infant of difficulty in swallowing and two episodes of severe well between episodes, except for continued spitting-up chusetts General Hospital, she was a vigorous infant in upper lobe pneumonia, confirmed by chest roentgenogram. gurgitation at the pharyngeal level of about half of each
was admitted because of recurrent bouts respiratory distress. The child appeared of feedings. On referral to the Massano distress but had clinical signs of right A tine barium swallow revealed reswallowed bolus, but that which passed
through the pharynx passed down to the esophagus normally. Palpation of the pharynx suggested a mass. Surgical consultation was sought on her tenth hospital day. On direct laryngoscopy, a cystic mass was observed at the base of the tongue. During this procedure, the child’s airway became completely obstructed, requiring emergency intubation. She was taken to the operating room immediately where tracheostomy was performed over a No. 3 bronchoscope. On repeat laryngoscopy in the operating room, a 3-cm mass arising from the fold between the base of the tongue and the epiglottis was unroofed (Fig. 2). The wall of the cyst was thick and contained thick mucoid material. Postoperatively, the child began taking feedings normally on the second day without respiratory
From the Division of Pediatric Surgery, Massachusetts General Hospital, and the Department of Surgery, Harvard Medical School, Boston, Mass. Presented before the 6th Annual Meeting of the American Pediatric Surgical Association. San Juan, Puerto Rico, April 10-12. 1975. Address for reprint requests: W. Hardy Hendren. M.D.. Massachusetts General Hospital, Boston. Mass. 02114. rQ1975 by Grune & Stratton. Inc. Journal of Pediatric Surgery, Vol. 10, No. 5 (October), 1975
807
CANTY
CASE
AND
HENDREN
I
Fig. 1. Case 1. (A) Diagrammatic representation of large hypopharyngeal lesion completely obstructing glottic inlet. (8) Photomicrograph showing an external mucosal lining of squamous epithelium, a thick middle layer of musculofibrous tissue, and an inner lining of respimtory epithelium. Note presence of subepithelial mucous glands.
Based
on tongue
/
Fig. 2. Caso 2. Diagmmmatic representation of cystic Ierion arising at base of tongue and obstructing glottic inlet.
FOREGUT
CYSTS
809
distress. Her tracheostomy tube was removed on the seventh postoperative day, and the child was discharged on the tenth day. Histologic examination of the cyst wall revealed both surfaces to be covered with squamous epithelium separated by a thick inner layer of skeletal muscle. This was consistent with an upper esophageal duplication.
Case 3 R.M. (MGH No. 177-03-88). a lo-day-old male infant was referred because of persistent upper airway obstruction and a mass in the posterior pharynx, encroaching on the larynx. Laryngoscopy under anesthesia revealed a large cystic mass arising in the left pyriform sinus, almost covering the glottic inlet (Fig. 3). Endotracheal intubation was performed. The cyst wall was unroofed with cautery, releasing thick, mucoid material. The child was extubated. breathed well, and tracheostomy was not needed, ReexaminaHe was well for 5 mo but then began to exhibit recurrence of respiratory distress. tion under anesthesia revealed recurrence of the cystic mass. It was unroofed again, relieving his symptoms. Symptoms recurred once again 1 mo later, again requiring laryngoscopy and intubation. On this occasion, the outer wall of the mass was extensively resected, unroofing it several centimeters downward to the level of the cricoid cartilage. The considerable extent of the lesion had not been appreciated at the first procedures. Histopathology of the cyst showed it to be thick-walled. lined with respiratory epithelium on one side and squamous epithelium on the other, separated by a thick, muscular layer. consistent with a bronchogenic foregut cyst,
Case 4 In contrast to these three cases of cysts of foregut origin is a fourth case of a simple laryngeal cyst that proved lethal to the infant. J. C. (MGH No. 120-77-35). a l-day-old male infant was referred because of upper airway obstruction. He was noted to be cyanotic at birth with crowing respirations. He was in severe respiratory distress on admission. Under anesthesia, laryngoscopy revealed a large cyst arising from the left laryngeal wall almost completely blocking the inlet, A bron-
Fig. 3.
Diagram of lesion in care 3.
810
CANM
AND HENDREN
Fig. 4. Case 4. (A) Photograph of larynx at autopsy showing cystic lesion arising in laryngeal inlet. (6) Photomicrograph of wall of simple cyst of larynx. Note thin wall composed of a few layen of simple stroma in contrast to thick muscular wall seen in Fig. 1 B.
choscope was then passed, and tracheostomy was performed using a silver metal tracheostomy tube. This event was prior to the era when present day plastic tubes were in use. The child did well for 5 days, then suddenly died. At postmortem examination, it was found that the tip of the metal tracheostomy tube had perforated the trachea. The large thin-walled cyst was noted within the left lateral wall of the larynx markedly narrowing the glottic inlet (Fig. 4A). Histopathologic examination of this showed a thin-walled cyst composed only of a single layer of lining cells and a thin fibrous capsule (Fig. 4B) This was considered to be a simple laryngeal cyst. DISCUSSION
Respiratory distress in.the newborn period secondary to apparent airway obstruction is most commonly due to congenital laryngeal stridor or laryngomalacia.3q4 This condition rarely requires surgical intervention and usually clears spontaneously in a few months. It is best recognized at direct laryngoscopy where one sees the floppy glottic folds that, on inspiration, are drawn in and partially occlude the airway. Other causes of airway obstruction include congenital webs and stenoses of the larynx; these often do not cause difficulties until later in infancy. Holinger reported congenital subglottic stenosis as the most common anomaly of the tracheobronchial tree requiring tracheostomy prior to 1 yr of age? Other lesions include lymphoma and hemangioma, which can impinge on the larynx from outside and cause respiratory distress. Cystic lesions of the larynx arising in the newborn are rare. The majority of these are laryngoceles arising from the ventricles of the larynx. These thinwalled, air-containing cysts may assume large size and cause airway obstruction in the newborn. Laryngoceles are best treated by immediate direct laryngoscopy and aspiration of the cyst. They may occasionally recur, requiring excision.“6
FOREGUT
CYSTS
811
Simple mucous cysts of the larynx, as typified by case 4, are also rare. Holinger reported only three cases in a 30-yr experience.4 De Santo et al. found 190 cases in the Mayo Clinic records of cysts of the larynx in all ages covering a 20-yr period.5 These cysts rarely caused life-threatening airway obstruction. Some believe these lesions to be simple retention cysts resulting from obstruction of mucous gland ducts. They may occur anywhere in the larynx from the aryepiglottic fold to the laryngeal ventricle and are best treated by transoral aspiration and partial excision of the cyst wall to prevent recurrence. When airway obstruction is severe, establishment of an adequate airway by emergency bronchoscopy and tracheostomy is necessary. Cysts of the type presented by cases 1, 2, and 3 are apparently quite rare. In 1940, Ladd and Gross described an 1 I-mo-old infant presenting with difficulty in swallowing secondary to a duplication cyst at the base of the tongue; this was excised without difficulty;’ it was a colonic duplication. Daudet, reporting in a symposium on gastrointestinal duplications in 1965, made mention of a personal communication regarding three infants with duplication cysts of the oropharynx.2 Respiratory distress, however, was not a presenting symptom in any of these cases. These thick-walled, multilayered, cystic lesions containing layers of muscle and organized epithelial coverings on both sides were classified by Ravitch as cysts of foregut origin.7 They are further divided into three categories: (I) Enteric cysts, which are lined by columnar or ciliated epithelum, frequently contain ectopic gastric mucosa, usually lie in the posterior mediastinum, and are a form of esophageal duplication. They are frequently associated with abnormalities of the vertebral bodies. (2) Bronchogenic cysts, which are lined by respiratory epithelium with a poorly developed muscle wall, commonly arise from the bronchi. (3) Esophageal cysts, often lying intramurally within the esophagus, are not associated with vertebral abnormalities. cases 1 and Our three cases are best classified as foregut cysts. Furthermore, 3 fit the bronchogenic cyst category, and case 2, the esophageal type. The histopathologic classification is of importance in regard to treatment. Simple aspiration of this type of cyst, as is done for laryngoceles and which was practiced for many years for mediastinal duplication cysts, is rarely successful.s Duplication cysts must be adequately unroofed or recurrence is common. This point is clearly underlined by our experience with case 3. It is puzzling to note that this report of foregut cysts in this location causing airway obstruction is unique in the literature. These lesions are most likely much more common than previously supposed. Careful histopathologic examination and classification of resected cystic lesions from this location will be necessary to reveal their true incidence. These four cases emphasize the importance of direct laryngoscopy and bronchoscopy for the immediate diagnosis and treatment of severe respiratory distress secondary to airway obstruction. Cystic lesions are rare causes of airway obstruction; however, the presenting symptoms of inspiratory stridor and sternal retraction should alert physicians to this possibility and prompt direct visualization of the oropharynx and larynx. Definitive treatment can then be readily carried out.
812
CANTY
AND HENDREN
REFERENCES I. Ladd WE, Gross RE: Surgical treatment of duplications of the alimentary tract. Surg Gynecol Obstet 70: 295-307, 1940 2. Daudet M: Symposium Consacre aux duplications intestinales. Ann Chir Infantile 8: 5-80, 1967 3. Shackelford GD, McAlister WH: Congenital laryngeal cyst. Am J Roentgenal 114: 289-292, 1972 4. Holinger PH. Brown WT: Congenital webs, cysts, laryngoceles and other anomalies of the larynx. Ann Otol Rhino1 Laryngol 76: 744-752, 1967
5. De Santo LW, Devine KD, Weiland LH: Cysts of the larynx-classification. Laryngoscope 80: 145-176, 1970 6. Suehs OW, Powel DB: Congenital cysts of the larynx in infants. Laryngoscope 77: 654-662, 1967 7. Ravitch MM, Sabiston DC: Mediastinal infections and tumors, in Mustard WT. Ravitch MM, Snyder WH Jr, et al (eds): Pediatric Surgery, Chicago, Year Book, 1969, pp 417-420 8. Bishop HC, Koop CE: Surgical management of duplications of the alimentary tract. Am J Surg 107: 434-442, 1964
