Congenital esophageal stenosis due to tracheobronchial remnants

Congenital Esophageal

Stenosis due to

Tracheobronchial By

MASANOBU

ISHJDA,

YOSHIAKI

Remnants TSUCHIDA,

SUMIO

SAITO,

AND AKIO TSUNODA

I

IMPLANTS of tracheobronchial remnants such as cartilage, tracheal glands, and respiratory epithelium have been recognized as a cause of congenital esophageal stenosis. This anomaly was first reported by Frey1 in 1936. Clinicopathologic study of the anomaly, however, has not been undertaken because of limited information. NTRAMURAL

CASE

REPORTS

Cuse 1: A 17-month-old, underdeveloped boy was admitted with a history of vomiting from the age of 7 months. A barium swallow showed abrupt narrowing of the extreme distal end of the esophagus with dilatation above it. Exploration via the abdomen demonstrated a firm stricture in the distal esophagus, just above the esophagogastric junction. There was cartilage in a frozen section of a firm nodule in the stricture. Esophagogastrostomy (Heyrovsky’s procedure) was performed. His postoperative course was complicated by hiatal herniation of the stomach and reflux esophagitis. After repair of the herniation, he recovered and was swallowing well when last seen one year after operation. In addition to cartilage, the resected specimen contained glands of tracheal origin (Fig. 1A) and stratified columnar ciliated epithelium surrounded by lymphoid tissue (Fig. 1B ) , quite similar to branchiogenic lymphepithelial tissue. Case 2: A 4-year-old boy had difficulty in swallowing which appeared when a change was made to a solid diet. The dysphagia was not severe and his physical development was within normal limits. On barium swallow there was narrowing of the lower end of the esophagus with considerable diIatation above, The diagnosis of achalasia was suspected, but a response to methacholine was negative. Exposure via laparotomy demonstrated two pieces of cartilage-like tissue on the esophageal wall 3 cm. above the esophagogastric junction. After biopsy, an esophagogastrostomy (Grondahl’s procedure) was done. The child recovered promptly and was swallowing well when last seen 3 years after the operation. The resected specimen was similar to that of Case 1, showing cartilage, tracheal glands, and lymphepithelial tissue. Case 3: The patient was a 5-year-old, underdeveloped boy with difficulty in swallowing which had appeared at the age of 1 year, There was abrupt narrowing of the distal esophagus with proximal dilatation on barium swallow. Exploration through the abdomen demonstrated a firm stricture in the distal end of the esophagus with a small cartilaginous mass. The distal esophagus was excised and continuity restored by esophagogastrostomy, after which the child did well. From The Second Department Tokyo, Tokyo, Japan.

of Surgery,

The

Faculty

of Medicine,

The

University

of

MASANOBU ISHIDA, M.D.: Associate Professor, The Second Department of Surgery, The Associate, The Second Department of University of Tokyo. YOSHIAKI TSUCHWA, M.D.: Surgery, The University of Tokyo. SUMIO SAITO, M.D.: Lecturer, The Seczmd Department of Surgery, The University of Tokyo. AKIO TSUNODA, M.D.: Associate, The Second Department of Surgery, The University of Tokyo. JOURNAL OF PEDIATRIC SURGERY, VOL. 4,

No.

3 (JUNE),

1969

339

340

ISHJDA ET AL.

Fig. 1 .-A, The resected specimen (Case 1) contained cartilage (left) and glands of tracheal origin (right). (H and E, X 360) B, Branchiogenic lymphepithelial tissue (Case 2). Stratified columnar ciliated epithelium, so-called respiratory epithelium, was surrounded by lymphoid tissue. (H and E X 360) Histologic examination showed cartilage and glands of tracheal origin. On the mucosa overlying the stricture, there was a tiny opening of a small, fistulous tract leading into the muscularis, lined microscopically with squamous epithelium and contiguous with stratified columnar ciliated epithelium and primordial lymphepithelial tissue (Fig. 2). DISCUSSION

Esophageal

stenosis

associated

with tracheobronchial

remnants

was first re-

ported in 1936 by Frey.1 He found microscopic cartilage in the cardia of a 19-

ESOPHAGEAL

STIXNOSIS DUE TO TBACHEOBRONCHIAL

REMNANTS

341

Fig. 2. On the mucosa overlying the stricture, there was a tiny opening of a small, fistulous tract (left) leading into the muscularis. This tract was lined with stratified squamous epithelium and was contiguous with stratified columnar ciliated epithelium and primordial lyrnphepithelial tissue (Case 3). (H and E X 120) year-old girl who died with a clinical diagnosis of cardiospasm. A similar case was reported from Massachusetts General Hospital in 1956.2 That patient was a 52-year-old man with difficulty in swallowing from the age of 3 years and there were respiratory-epithelium-lined cyst, fragments of cartilage, and tracheal glands in the distal third of the esophagus. Kumar,3 in 1962, and Paulino,4 in 1963, reported such cases in infancy. In the Japanese literature there have been several reports since that of Miwa5 in 1957. Yanagisawa6 reported the largest group of 4 cases in 1965. Cases reported in the English and German literature are listed in Table 1. Including our 3 cases, the ratio of females to males is 4:4. The onset of symptoms was in infancy or childhood, even though ages on admission were widely distributed. In almost all patients, cartilage and other tracheobronchial remnants were in the distal end of the esophagus, located intramuscularly and sometimes protruding externally. Bergmann,’ in 1958, and Spath,8 in 1959, reported instances of esophageal stenosis associated with mucous glands and respiratory epithelium, but not associated with cartilage deposit. These cases are closely related to the anomaly reported here and included in Table 1. Embryology It is well known that the trachea begins to separate from the esophagus when the embryo is about 4 mm. long and the entire process of separation is completed by the 17 mm. stage .O The anomaly reported here has been considered to result from failure of the normal embryonic separation of the respiratory tract from the esophagus.“-4 The following anomalies presumably belong to the same category. 1. Patches of ciliated epithelium in the adult esophagus.10 2. Esophageal cyst lined by ciliated epithelium.ll*l” 3. Esophageal stenosis due to tracheobronchial remnants other than cartilage.‘T8

19 52 10 8 14 17 4 5

yrs. yrs. mos. mos. mos. mos. yrs. yrs.

49 yrs.

57 yrs.

Age at Onset

F M F F F M M M

F

F

? yrs. mos. mos. mos. mos. mos. yr.

Distal end 2.5 cm. above car&a Distal end Lower third Distal end Distal end Distal end Distal end

0 mos.

childhood

-

4 f .b. above cardia

:

:

Cartilage

+

+

::

+

Histologic Mucous Glands

Remnants (Reported Cases)

10 cm. above diaphragm

Remnants other than Cartilage:

3 4 0 6 7 6 1

Remnants:

Site of Stenosis

Stenosis Due to Tracheobronchial

and Other Tracheobronchial

sex

Stenosis due to Tracheobronchial

(1958)

Spaths (1959)

Bergmann’

II. Esophageal

Ishida ( 1968)

Age

Table l.-Esophageal

Stenosis due to Cartilage

Frey1 (1936) M. G. H.2 (1956) Kumar3 (1962) Paulino4 ( 1963 )

I. Esophageal

Authors

epithelium

Others

Respiratory epithelium with lymphoid tissue Bronchial epithelium with lymphoreticular tissue

Respiratory epithelium Respiratory epithelium Lymphepithelial tissue Lymphepithelial tissue

Respiratory

Findings

ESOPHAGEAL

STENOSIS

DUE

TO TBACHEOBRONCHIAL

REMNANTS

343

4. Esophageal stenosis due to cartilage and other tracheobronchial remnants.lW6 5. Accessory lung arising from the esophagus.13s14 The occurrence of cartilage in the lower esophagus has been described also in a dromedary fetus.15 Pathology

Cartilage is apparently of prime importance in causing esophageal stenosis. Cartilage deposits were often so large as to be described as cartilaginous ring.3*4 Occasionally, they were so small that they could be found only by microscopic examination.2s6 Mucous glands, regarded as of tracheal origin,” associated with cartilage were recorded in almost all cases. The presence of respiratory epithelium was also reported by Paulino4 and others .2 However, it is not justified to term it “respiratory epithelium” or “bronchial epithelium.“16s17 According to Johns,l’ stratified coIumnar ciIiated epitheIium appears in the embryonal esophagus at the 40 mm. stage and comprises an almost complete esophageal lining up to the 130 mm. stage. Patches of ciliated epithelium may be present at birth.17p1* So, the presence of ciliated epithelium in this anomaly can be regarded as a localized arrest in development with persistence of the embryonic esophageal epithelium. In two instances reported here, stratified columnar cihated epithelium was surrounded by lymphoid tissue. The same histologic finding was reported by Bergmann’ as “respiratory epithelium surrounded by lymphoid follicles.” Histologic examination of our specimens revealed that it is not an inflammatory reaction but a kind of lymphepithelial tissue. It closely resembles branchiogenic lymphepithelial tissues such as the tonsil, the thyroglossal remnants, the thymicopharyngeal remnants, and so forth, although some of these branchiogenic lymphepithelial tissues contain stratified squamous epitheIium.lg As the tracheobronchial groove, from which the respiratory system arises, is located immediately caudal to the fourth pharyngeal pouch and may represent fused pharyngeal pouches, 2o it may be justifiable to propose the term “branchiogenic IymphepitheliaI tissue.” It is unknown why these lymphepithelial tissues are associated with cartilage, but it is of embryologic interest that the tonsil-a branchiogenic lymphepithelial tissue-sometimes contains cartiIage.16s21 It may be presumed that mesenchymal cells, which form cartilage, become sequestrated in the esophagus, causing a localized arrest of development in the esophageal epithelium and consequent formation of the lymphepithelial tissue. The other possible explanation is a localized arrest of development of the esophagus. Persistence of the embryonic epithelium is presumably followed by lymphepithelial tissue formation and a possible metaplasia of the mesenchymal cells produces cartilage. The largest lymphepithelial tissue in this series was 8 mm. X 5 mm., large enough to cause esophageal stenosis. Cases of esophageal stenosis due only to this lymphepithelial tissue were reported by Bergmann in 1958 and Spatha in 1959; none of their specimens contained cartilage.

344

ISHIDA ET AL.

When the tracheobronchial remnants are small as compared with the caliber of the esophageal tract, the cause of obstruction is presumably interruption of progressive esophageal peristalsis. On the other hand, it might be simply mechanical, if the cartilage is so large as to be described as “cartilaginous ring.‘3r4 SUMMARY

cases of esophageal stenosis due to intramural tracheobronchial remnants are reported. Cartilage, tracheal glands, and brachiogenic lymphepithelial tissue were found in the distal end of the esophagus. Esophageal stenosis due to these tracheobronchial remnants, although rare, accounts for some congenital distal esophageal stenosis in infancy. Branchiogenic lymphepithelial tissue, here first reported, can cause the esophageal stenosis as well as cartilage. Three

SUMMARIO IN INTERLINGUA Stenose esophagee causate per remanentes tracheobronchial es un rar sed distincte entitate clinic in i&antes. Tres tal cases es addite al 7 jam reportate. Cartilagine, glandulas tracheal, e branchiogenic tissu lymphepithelial esseva trovate in le termino distal de1 esophago. Es concludite que non sohnente cartilagine sed etiam branchiogenic tissu lymphepithelial (prim0 reportate in iste case) pote causar stenose esophagee. REFERENCES 1. Frey, E. K., and Duschl, L.: Der Kardiospasmus. Ergebn. Chir. Orthop. 29: 637, 1936. 2. Case 42411, Abortive congenital esophago-tracheal fistula. Case records of the Massachusetts General Hospital. New Eng. J. Med. 255:707, 1956. 3. Kumar, R.: A case of congenital esophageal stricture due to a cartilaginous ring. Brit. J. Surg. 49:533, 1962. 4. Pa&no, F., Rose&, A., and Aprigliano, F.: Congenital esophageal stricture due to tracheobronchial remnants. Surgery 53:547, 1963. 5. Miwa, A., and Kitamura, S.: A case of congenital esophageal stenosis. Geka (Tokyo) 19: 1016, 1957. 6. Yanagisawa, F.: Achalasia in children, 4cta Pediat. Jap. (Tokyo) 67:131, 1965. 7. Bergmann, M., and Chamas, Ft. M.: I’racheobronchial rests in the esophagus. J. Thorac. Surg. 35:97, 1958. M.: 8. Spath, v. F., and Ratzenhofer, Ueber die angeborene Oesophagusstenose. Wien. Klin. Wschr. 71:723, 1959. 9. Arey, L. B.: Developmental Anatomy. Philadelphia, W. B. Saunders Co., 1936. 10. Raebum, C.: Columnar ciliated epithelium in the adult esophagus. J. Path. Bact. 63:157, 1951.

11. Totten, R. S., Stout, A. P., Humphreys, G. H., Jr., and Moore, R. L.: Benign tumors and cysts of the esophagus. J. Thorac. Surg. 25:606, 1953. 12. Case 33182, Congenital tracheobronchial cyst of the esophageal wall. Case records of the Massachusetts General Hospital. New Eng. J. Med. 236:672, 1947. 13. Gans, S. L., and Potts, W. J.: Anomalous lobe of lung arising from the esophagus. J. Thorac. Surg. 21:313, 1951. 14. Boyden, E. A., Bill, A. H., and Creighton, S. A.: Presumptive origin of a left lower accessory lung from an esophageal diverticulum. Surgery 52:323, 1962. 15. Maskar, U.: Ueber eine Knorpelanlage im Oesophagus eines Dromedarfetus. Acta Anat. 44:206, 1961. of 16. Willis, R. A.: The Borderland Embryology and Pathology. London, Butterworth and Co. Ltd., 1962. 17. Johns, B. A. E.: Developmental changes in the esophageal epithelium in man. J. Anat. 86:431, 1952. 18. Rector, L. E., and Connerley, M. L.: Aberrant mucosa in the esophagus in infants and children. Arch. Path. 31:285, 1941. 19. Curran, R. C.: Colour Atlas of Histopathology. London, Bailliere, Tindall and Cassell, 1966.

ESOPHAGEAL

STENOSIS

DUE

TO

TBACHEOBRONCHIAL

20. Hamilton, W. J., Boyd, J. D., and Mossman, H. W.: Human Embryology. Cambridge, W. Heffer and Sons Ltd., 1959.

REMNANTS

21. Ruckert, Knorpelbefunde Arch. 177:387,

345

A.: Ueber Knochen und in den Tonsllen. Virchow. 1904.