- Email: [email protected]
Esophageal stenosis due to cartilaginous tracheobronchial remnants
Esophageal Stenosis Due to Cartilaginous Tracheobronchial Remnants By W. F. Sneed, D. C. LaGarde, M. S. Kogutt, and R. M. Arensman
N e w Orleans, Louisiana 9 In cases of congenital esophageal stenosis, tracheobronchial remnants in the distal esophagus should be considered a possible diagnosis. The cause is thought to be esophageal sequestration of a tracheobronchial anlage before embryologic separation. Primary resection of the stenotic portion of the esophagus with re-anastomosis is recommended. Such a case is reported. INDEX WORDS: Esophageal stenosis; tracheobronchial remnants.
A
C O M M O N ETIOLOGY of esophageal stenoses is reflux esophagitis with peptic stricture. Less likely possibilities are idiopathic fibrous or muscular stenosis or esophageal web. Even more remote is the possibility of ectopic tissue, such as tracheobronchial remnants resulting in encircling cartilaginous rings. The details of such a case are presented and compared to the 21 previous cases reported.
resection of the stenotic area with an end-to-end esophagogastrostomy and Nissen fundoplication was performed. Histologic examination of the specimen revealed tracheobronchial remnants including hyaline cartilage within the muscular layers of the esophageal wall, and multiple seromucous glands lined with respiratory epithelium (Fig. 2). Eight months postoperatively, the patient is tolerating a regular diet and barium passes freely through the anastomotic site into the stomach.
DISCUSSION
Many of the reported cases of congenital esophageal stenosis are inflammatory strictures secondary to reflux esophagitis. However, three
CASE REPORT A white female infant was well until 6 mo of age when she began to vomit her formula. Because of progressive frequency of vomiting, she was evaluated by her local pediatrician at 13 mo of age. An upper gastrointestinal series revealed a stenosis of the distal esophagus. Although the diagnosis of achalasia was entertained, no specific therapy was instituted. When symptoms worsened and the infant became progressively malnourished, she was referred to our institution. At initial examination the patient was 20 mo old and weighed 9.2 kg (third percentile). An upper gastrointestinal examination showed stenosis of the lower esophagus with severe proximal dilatation (Fig. 1). No esophageal reflux or hiatus hernia was demonstrated. Esophagoscopy revealed a firm stenotic lesion that was rigid and unyielding. Biopsy demonstrated only minimal atypia of the esophageal mucosa. At laparotomy retrograde dilation failed, therefore local
From the Departments of Pediatric Surgery, Pediatrics, and Radiology, Ochsner Medical Institutions, New Orleans, La. Presented before the lOth Annual Meeting of the American Pediatric Surgical Association, Los Angeles, California, March 25-28, 1979. Address reprint requests to Dr. Arensman, Ochsner Clinic, 1514 Jefferson Highway, New Orleans, La. 70121. 9 1979 by Grune & Stratton, Inc. 0022-3468/79/t 406-0030501.00/0 786
Fig. 1. Barium esophagram shows narrowing of the distal esophagus with proximal dilation.
Journal of Pediatric Surgery, Vol. 14, No. 6 (December),1979
ESOPHAGEAL STENOSIS
e
787
embryonic respiratory tract from the primitive esophagus, resulting in sequestration of some tracheobronchial precursor cells within the wall of the esophagus. Esophageal stenosis resulting from tracheobronchial remnants was first reported by Frey and Duschel ~ in 1936, when cartilage was found in the distal esophagus of a 19-year-old girl who had died with the diagnosis of achalasia. Since then, 21 cases of stenosis secondary to tracheobronchial remnants have been reported j ~3 (Table 1). Although the age at diagnosis varied from 1 day to 57 yr in the reported cases, symptoms of esophageal obstruction appeared in infancy or childhood in all cases. With one exception, 9 the stenosis has occurred in the distal third of the esophagus. An esophagram, showing a stenosis of the distal esophagus with proximal dilation, is the most useful diagnostic measure. At endoscopy, the esophageal mucosa appears normal, and biopsy confirms the absence of esophagitis. Unlike achalasia and esophageal webs, dilation of the stenosis is usually unsuccessful and
.-.~":.
2~"~
(
.
.
.
~;&
.!
....
Fig. 2. Histologic section from stenotic area of esophagus shows cartilage, abundant respiratory mucous glands, and ciliated respiratory epithelium (hematoxylin and eosin x 35).
types of congenital esophageal stenosis without reflux have been described: ( 1) segmental hypertrophy of the muscularis and submucosal layers of the esophageal wall; (2) membranous web or diaphragm; and (3) intramural rests of tracheobronchial tissue. The last type, which our patient had, is generally considered to be a result of a disturbance in normal separation of the Table 1.
Author
Age
Frey (1936)
19 yr
M.G.H. (1947)
4 3 yr
Sex
Age at Onset of Sx
Histolegic Components
Sire of Steeosis Distal end
Cartilage +
Glands
Resp. Epithelium
+
Operative Method Diagnosed at postmortem examination
M
28 yr
?
-
M.G.H. (1956)
52 yr
M
Bergmann (1958)
57 yr
F
Childhood
Lower third
Spath (1959)
49 yr
F
Birth
Lower third
Kumar (1962)
10 me
F
Pauline (1963)
8me
F
14 me
F
17 me
M
4
M
6 me
Distal end
5
M
1 yr
Distal end
1 day
F
Upper third
+
Ishida (1969)
Goldman (1972)
3 yr
4 me
Distal end
+
+
Extramucosal excision
+
+
Resection
+
+
Resection
4
+
Resection
+
Resection
Distal end
4-
Lower third
+
6 me
Distal end
+
+
+
Extramucosal excision
7 me
Distal end
+
+
+
Resection
+
4
§
+
+
Resection
+
Cervical esophagostomy
Birth
Birth
+
Resection
Resection
(atresia) Fonkalsrud (1972)
1 yr
F
6 me
Lower third
Anderson (1973)
8 yr
F
7me
Distal end
+
Deiraniya (1974)
2 yr
F
6me
Lower third
+
+
Resection (with colon
10 me
M
5 me
Lower third
+
+
Diagnosed at postmortem
4 yr
M
18 me
Lower third
+
+
+
Resection
+
Resection interposition) examination
Ohkawa (1975)
Present case (1978)
Resection
20 me
M
9 me
Distal end
+
+
+
Resection
19 me
M
4 me
Distal end
+
+
+
Resection
4 yr
M
8 me
DistaI end
+
+
?
Resection
13 me
F
7 me
Distal end
4
+
?
Myomectomy
2 0 me
F
6 me
Distal end
+
+
+
Resection
788
SNEED ET AL.
h a z a r d o u s . D e i r a n i y a L2 r e p o r t e d a c a s e in w h i c h e s o p h a g e a l p e r f o r a t i o n followed d i l a t i o n o f a s t r i c t u r e s e c o n d a r y to i n t r a m u r a l t r a c h e o b r o n chial r e m n a n t s . S u r g i c a l excision o f the s t e n o t i c s e g m e n t , c o u p l e d with an a n t i r e f l u x p r o c e d u r e if the g a s t r o e s o p h a g e a l j u n c t i o n is r e m o v e d , is t h e r e c o m m e n d e d t r e a t m e n t . C o n t i n u i t y can u s u a l l y be r e - e s t a b l i s h e d by e n d - t o - e n d a n a s t o m o s i s .
REFERENCES 1. Frey EK, Duschel L: Der kardiospasmus. Ergeb Chirur Orthop 29:637-716, 1936 2. Case Records of the Massachusetts General Hospital. Case 33182. Congenital tracheobronchial cyst of the esophageal wall. N Engl J Med 236:172-674, 1974 3. Case Records of the Massachusetts General Hospital. Case 4241 I. N Engl J Med 255:707-710, 1956 4. Bergmann M, Charnas RM: Tracheobronchial rests in the esophagus; their relation to some benign strictures and certain types of cancer of the esophagus. J Thorac Surg 35:97 104, 1958
5. Spath F, Ratzenhofer M: Ueber die angeborene Oesophagusstenose. Wien Klin Wochenschr 71:723-727, 1959 6. Kumar R: A case of congenital oesophageal stricture due to a cartilaginous ring. Br J Surg 49:533-534+ 1962 7. Paulino F, Roselli A, Aprigliano F: Congenital esophageal stricture due to tracheobronchia] remnants. Surgery 53:547-550, 1963 8. Ishida M, Tsuchida Y, Saito S, et al: Congenital esophageal stenosis due to tracheobronchial remnants. J Pediatr Surg 4:339-345, 1969 9. Goldman RL, Ban JL: Chondroepithelial choristoma of the esophagus associated with esophageal atresia. J Thorac Cardiovasc Surg 63:318 321, 1972 10. Fonkalsrud EW: Esophageal stenosis due to tracheobronchial remnants. Am J Surg 124:101-103, 1972 11. Anderson LS, Shackelford GD, Mancilla-Jimenez R, et ah Cartilaginous esophageal ring: A cause of esophageal stenosis in infants and children. Radiology 108:665 666, 1973 12. Deiraniya AK: Congenital oesophageal stenosis due to tracheobronchial remnants. Thorax 29:720-725, 1974 13. Ohkawa H, Takahashi H, Hoshino Y, et ah Lower esophageal stenosis in association with tracheobronchial remnants. J Pediatr Surg 10:453-457, 1975
N e w Orleans, Louisiana 9 In cases of congenital esophageal stenosis, tracheobronchial remnants in the distal esophagus should be considered a possible diagnosis. The cause is thought to be esophageal sequestration of a tracheobronchial anlage before embryologic separation. Primary resection of the stenotic portion of the esophagus with re-anastomosis is recommended. Such a case is reported. INDEX WORDS: Esophageal stenosis; tracheobronchial remnants.
A
C O M M O N ETIOLOGY of esophageal stenoses is reflux esophagitis with peptic stricture. Less likely possibilities are idiopathic fibrous or muscular stenosis or esophageal web. Even more remote is the possibility of ectopic tissue, such as tracheobronchial remnants resulting in encircling cartilaginous rings. The details of such a case are presented and compared to the 21 previous cases reported.
resection of the stenotic area with an end-to-end esophagogastrostomy and Nissen fundoplication was performed. Histologic examination of the specimen revealed tracheobronchial remnants including hyaline cartilage within the muscular layers of the esophageal wall, and multiple seromucous glands lined with respiratory epithelium (Fig. 2). Eight months postoperatively, the patient is tolerating a regular diet and barium passes freely through the anastomotic site into the stomach.
DISCUSSION
Many of the reported cases of congenital esophageal stenosis are inflammatory strictures secondary to reflux esophagitis. However, three
CASE REPORT A white female infant was well until 6 mo of age when she began to vomit her formula. Because of progressive frequency of vomiting, she was evaluated by her local pediatrician at 13 mo of age. An upper gastrointestinal series revealed a stenosis of the distal esophagus. Although the diagnosis of achalasia was entertained, no specific therapy was instituted. When symptoms worsened and the infant became progressively malnourished, she was referred to our institution. At initial examination the patient was 20 mo old and weighed 9.2 kg (third percentile). An upper gastrointestinal examination showed stenosis of the lower esophagus with severe proximal dilatation (Fig. 1). No esophageal reflux or hiatus hernia was demonstrated. Esophagoscopy revealed a firm stenotic lesion that was rigid and unyielding. Biopsy demonstrated only minimal atypia of the esophageal mucosa. At laparotomy retrograde dilation failed, therefore local
From the Departments of Pediatric Surgery, Pediatrics, and Radiology, Ochsner Medical Institutions, New Orleans, La. Presented before the lOth Annual Meeting of the American Pediatric Surgical Association, Los Angeles, California, March 25-28, 1979. Address reprint requests to Dr. Arensman, Ochsner Clinic, 1514 Jefferson Highway, New Orleans, La. 70121. 9 1979 by Grune & Stratton, Inc. 0022-3468/79/t 406-0030501.00/0 786
Fig. 1. Barium esophagram shows narrowing of the distal esophagus with proximal dilation.
Journal of Pediatric Surgery, Vol. 14, No. 6 (December),1979
ESOPHAGEAL STENOSIS
e
787
embryonic respiratory tract from the primitive esophagus, resulting in sequestration of some tracheobronchial precursor cells within the wall of the esophagus. Esophageal stenosis resulting from tracheobronchial remnants was first reported by Frey and Duschel ~ in 1936, when cartilage was found in the distal esophagus of a 19-year-old girl who had died with the diagnosis of achalasia. Since then, 21 cases of stenosis secondary to tracheobronchial remnants have been reported j ~3 (Table 1). Although the age at diagnosis varied from 1 day to 57 yr in the reported cases, symptoms of esophageal obstruction appeared in infancy or childhood in all cases. With one exception, 9 the stenosis has occurred in the distal third of the esophagus. An esophagram, showing a stenosis of the distal esophagus with proximal dilation, is the most useful diagnostic measure. At endoscopy, the esophageal mucosa appears normal, and biopsy confirms the absence of esophagitis. Unlike achalasia and esophageal webs, dilation of the stenosis is usually unsuccessful and
.-.~":.
2~"~
(
.
.
.
~;&
.!
....
Fig. 2. Histologic section from stenotic area of esophagus shows cartilage, abundant respiratory mucous glands, and ciliated respiratory epithelium (hematoxylin and eosin x 35).
types of congenital esophageal stenosis without reflux have been described: ( 1) segmental hypertrophy of the muscularis and submucosal layers of the esophageal wall; (2) membranous web or diaphragm; and (3) intramural rests of tracheobronchial tissue. The last type, which our patient had, is generally considered to be a result of a disturbance in normal separation of the Table 1.
Author
Age
Frey (1936)
19 yr
M.G.H. (1947)
4 3 yr
Sex
Age at Onset of Sx
Histolegic Components
Sire of Steeosis Distal end
Cartilage +
Glands
Resp. Epithelium
+
Operative Method Diagnosed at postmortem examination
M
28 yr
?
-
M.G.H. (1956)
52 yr
M
Bergmann (1958)
57 yr
F
Childhood
Lower third
Spath (1959)
49 yr
F
Birth
Lower third
Kumar (1962)
10 me
F
Pauline (1963)
8me
F
14 me
F
17 me
M
4
M
6 me
Distal end
5
M
1 yr
Distal end
1 day
F
Upper third
+
Ishida (1969)
Goldman (1972)
3 yr
4 me
Distal end
+
+
Extramucosal excision
+
+
Resection
+
+
Resection
4
+
Resection
+
Resection
Distal end
4-
Lower third
+
6 me
Distal end
+
+
+
Extramucosal excision
7 me
Distal end
+
+
+
Resection
+
4
§
+
+
Resection
+
Cervical esophagostomy
Birth
Birth
+
Resection
Resection
(atresia) Fonkalsrud (1972)
1 yr
F
6 me
Lower third
Anderson (1973)
8 yr
F
7me
Distal end
+
Deiraniya (1974)
2 yr
F
6me
Lower third
+
+
Resection (with colon
10 me
M
5 me
Lower third
+
+
Diagnosed at postmortem
4 yr
M
18 me
Lower third
+
+
+
Resection
+
Resection interposition) examination
Ohkawa (1975)
Present case (1978)
Resection
20 me
M
9 me
Distal end
+
+
+
Resection
19 me
M
4 me
Distal end
+
+
+
Resection
4 yr
M
8 me
DistaI end
+
+
?
Resection
13 me
F
7 me
Distal end
4
+
?
Myomectomy
2 0 me
F
6 me
Distal end
+
+
+
Resection
788
SNEED ET AL.
h a z a r d o u s . D e i r a n i y a L2 r e p o r t e d a c a s e in w h i c h e s o p h a g e a l p e r f o r a t i o n followed d i l a t i o n o f a s t r i c t u r e s e c o n d a r y to i n t r a m u r a l t r a c h e o b r o n chial r e m n a n t s . S u r g i c a l excision o f the s t e n o t i c s e g m e n t , c o u p l e d with an a n t i r e f l u x p r o c e d u r e if the g a s t r o e s o p h a g e a l j u n c t i o n is r e m o v e d , is t h e r e c o m m e n d e d t r e a t m e n t . C o n t i n u i t y can u s u a l l y be r e - e s t a b l i s h e d by e n d - t o - e n d a n a s t o m o s i s .
REFERENCES 1. Frey EK, Duschel L: Der kardiospasmus. Ergeb Chirur Orthop 29:637-716, 1936 2. Case Records of the Massachusetts General Hospital. Case 33182. Congenital tracheobronchial cyst of the esophageal wall. N Engl J Med 236:172-674, 1974 3. Case Records of the Massachusetts General Hospital. Case 4241 I. N Engl J Med 255:707-710, 1956 4. Bergmann M, Charnas RM: Tracheobronchial rests in the esophagus; their relation to some benign strictures and certain types of cancer of the esophagus. J Thorac Surg 35:97 104, 1958
5. Spath F, Ratzenhofer M: Ueber die angeborene Oesophagusstenose. Wien Klin Wochenschr 71:723-727, 1959 6. Kumar R: A case of congenital oesophageal stricture due to a cartilaginous ring. Br J Surg 49:533-534+ 1962 7. Paulino F, Roselli A, Aprigliano F: Congenital esophageal stricture due to tracheobronchia] remnants. Surgery 53:547-550, 1963 8. Ishida M, Tsuchida Y, Saito S, et al: Congenital esophageal stenosis due to tracheobronchial remnants. J Pediatr Surg 4:339-345, 1969 9. Goldman RL, Ban JL: Chondroepithelial choristoma of the esophagus associated with esophageal atresia. J Thorac Cardiovasc Surg 63:318 321, 1972 10. Fonkalsrud EW: Esophageal stenosis due to tracheobronchial remnants. Am J Surg 124:101-103, 1972 11. Anderson LS, Shackelford GD, Mancilla-Jimenez R, et ah Cartilaginous esophageal ring: A cause of esophageal stenosis in infants and children. Radiology 108:665 666, 1973 12. Deiraniya AK: Congenital oesophageal stenosis due to tracheobronchial remnants. Thorax 29:720-725, 1974 13. Ohkawa H, Takahashi H, Hoshino Y, et ah Lower esophageal stenosis in association with tracheobronchial remnants. J Pediatr Surg 10:453-457, 1975