1975, British Journal of Radiology, 48,16-18
Congenital oesophageal strictures due to cartilaginous rings By Judith S. Rose, M.D., E. G. Kassner, M.D., K. H. Jurgens, M.D., and J. Farman, M.B., Ch.B., M.Med. Departments of Radiology and Pathology, Downstate Medical Center, Brooklyn, New York (Received December, 1973)
and fever. Past history included multiple bouts of vomiting and recurrent respiratory infections since birth. Multiple features of trisomy 21, including microcephaly, mongoloid facies, bilateral cataracts, and retarded mental and physical development were present. A barium swallow disclosed oesophageal dilatation proximal to a 4 cm narrowing of the distal oesophagus adjacent to the cardia. Projecting from the medial wall of the midportion of this stricture was a 0-5 cm collection of barium suggestive of an ulceration (Fig. 3). A firm unyielding stenosis was encountered 31 cm below the incisors at oesophagoscopy. The patient was referred for surgery and the stenosis was excised. The patient graduated from a liquid to semi-solid diet without difficulty. She was, however, found dead in bed two weeks post-operatively and the cause of death was ascribed to aspiration.
ABSTRACT
Two cases of distal oesophageal stricture secondary to tracheo-bronchial remnants containing cartilage are presented. The clinical histories and radiographic appearances of these lesions are discussed as is the postulated embryogenesis. A characteristic finding, when present, is the linear collection of barium projecting horizontally from the area of stricture, representing respiratory epithelial ducts. Tracheobronchial cartilaginous rests are rarely found in the distal oesophagus, but when present may be responsible for congenital narrowing. This anomaly is usually encountered in the neonate or young infant when symptoms of dysphagia, vomiting and aspiration pneumonia occur (Kumar, 1962; Paulino, Roselli and Aprigliano, 1963). Sometimes symptoms may be unrecognized in the younger patient; a number of cases have been reported in adults (Bergmann and Charnas, 1958; Castleman, 1956; Frey and Duschl, 1936; Spath and Ratzenhofer, 1959). CASE REPORTS
Case 1 M.B., a seven-month old infant was admitted to Kings County Hospital in July 1971 with a two-day history of difficulty in swallowing. A barium study demonstrated dilatation of the oesophagus with persistent narrowing of the distal segment measuring 2 cm in length just proximal to the oesophagogastric junction (Fig. 1). At oesophagoscopy, several pieces of cardboard were wedged in the distal oesophagus and were removed. Four months later, the infant was readmitted with a sixday history of dysphagia and regurgitation of both liquids and solids. Several pieces of newspaper were present in the pharynx and these were removed manually. Nasogastric feedings were instituted and a repeat oesophagram disclosed that the radiographic appearances were unchanged. The patient was referred for surgery and the distal stenosed oesophagus was excised and an end-to-end anastomosis effected. The post-operative course was uneventful and oesophagram revealed a satisfactory patent anastomosis. Macroscopic description of specimen The resected strictured segment showed a number of firm, grey bands running circumferentially in the thickened wall. Microscopic description of specimen The lumen was lined by stratified squamous epithelium. The submucosa and muscularis were not well organized and contained mucus glands with ducts connecting with the oesophageal lumen. Several cartilage bands were located in the muscularis; the largest extended for nearly half the circumference of the wall. Ductlike structures with pseudostratified columnar epithelium, surrounded by dense aggregates of lymphocytes, were located both internal and external to the cartilage rings (Fig. 2). Case 2 L.H., an eight-year old female was first admitted to Kings County Hospital with a two-day history of cough 16
FIG. 1. Case 1. Barium swallow demonstrating the lower oesophageal stricture with dilatation above.
JANUARY
1975
Congenital oesophageal strictures due to cartilaginous rings
FIG. 2. Photomicrography showing a cross section of the oesophagus. (H&ExlO) L = Lumen C = Cartilage bands G = Glands D = Ducts E = Pseudostratified columnar epithelium surrounded by lymphoid aggregates.
Macroscopic description of specimen
A 3 X 2-5 X 0-5 cm segment of oesophagus was examined. It was bony hard in consistency and the mucosal surface had a pinkish-grey colour. There was no evidence of ulceration, but a 0 5 cm tract was present and extended from the medial aspect of the stenosed segment. Microscopic description of specimen
The oesophageal lumen was lined by stratified squamous mucosa and there was a disorganized submucosa and muscularis which contained numerous secreting glands and scattered mixed glands. Several cartilage plates and ductlike structures lined by ciliated pseudostratified columnar epithelium were present. DISCUSSION
Congenital oesophageal stenosis due to tracheobronchial remnants was first described in a 19-year17
FIG. 3. Case 2. Frontal view of a barium swallow demonstrating a markedly dilated oesophagus with a stenosed distal segment. There is a 0-5 cm tract extending medially from the strictured portion of the oesophagus.
old female by Frey and Duschl. The second case reported was also in an adult, a patient who was 52 years old with a history of intermittent dysphagia since the age of three (Castleman, 1956). While several cases in adults have been reported (Bergmann and Charnas, 1958; Spath and Ratzenhofer, 1959) the majority of patients are children whose ages range from eight months to five years when first diagnosed (Ishida et al., 1969). No sex predilection exists (Ishida et al., 1969). These anomalies have been presumed to result from a failure of normal embryonic separation of the respiratory tract from the primitive oesophagus. Other developmental disorders with tracheobronchial remnants probably belong to this category:
VOL.
48, No. 565 Judith S. Rose, E. G. Kassner, K. H. Jurgens and J. Farman
(1) tracheoesophageal fistula; (2) cysts lined by bronchial epithelium, whether mediastinal, pulmonary or intramurally situated in the oesophagus; and (3) diverticula of the oesophagus containing tracheobronchial tissue (Bergmann and Charnas, 1958; Ishida «**/., 1969). The tracheobronchial groove, the primordium of the respiratory system develops as a median ventral diverticulum of the foregut. This groove is intimately related to the oesophagus which develops from the foregut caudal to the respiratory diverticulum. Lateral indenting grooves form on both sides, eventually separating the respiratory primordium from the oesophagus (Arey, 1954). Kumar (1962) and Paulino et al. (1963), have hypothesized that tracheal cartilage sequestrates in the oesophagus before the two tubes have completely separated from each other. Since this cartilage is usually found in the distal oesophagus, these cells may well have been carried down the oesophagus during the normal process of growth. Many of the reported cases of congenital stenosis of the oesophagus have both cartilage and mucous glands in the stenotic segment (Ishida et al., 1969). Respiratory-like epithelium has commonly been identified in this abnormality (Castleman, 1956; Paulino et al., 1963) and it may be surrounded by lymphoid tissue (Bergmann and Charnas, 1958; Ishida et al., 1969). The origin of these lymphoid structures is unexplained. The radiographic appearance in our two patients was indicative of a non-neoplastic stricture of the distal oesophagus with secondary proximal dilatation. In our second case, there was a tiny collection of barium projecting medially from the midportion of the oesophageal narrowing. This was an important radiological finding in the evaluation of the stricture. Similar appearances have been described in the literature (Castleman, 1956; Kumar, 1962; Spath and Ratzenhofer, 1959). The significance of this finding, however, was not fully appreciated and at times was wrongly attributed to ulceration within the stricture. Pathologically, these barium-filled projections have proved to be ducts communicating with the oesophageal lumen. Multiple bilateral projections have been observed (Castleman, 1956; Spath and Ratzenhofer, 1959). The differential diagnosis of these congenital strictures includes stenoses secondary to ingestion of caustic agents, peptic oesophagitis with or without hiatus hernia, and less commonly achalasia. Caustic agents such as lye (Caffey, 1972) or
clinitest tablets (Genieser and Becker, 1969) can damage the oesophageal mucosa resulting in fibrosis and stricture. Corrosive strictures tend to be long in configuration and the history and initial endoscopic findings are diagnostic. Ingestion of clinitest tablets produces a circumferential narrowing, most often seen at the level of the aortic knuckle (Genieser and Becker, 1969). Reflux oesophagitis is frequently associated with hiatus hernia and is another cause of oesophageal stricture (Astley, 1956; Astley and Carre, 1954; Mustard et al., 1969). Ulcerations progress to fibrosis and tight stricture formation. In most instances the differential diagnosis is made by correlation of the history, endoscopic findings and early radiographic appearance of this lesion. Achalasia in the paediatric population is rare, especially in patients under one year of age (Magilner and Isard, 1971). The tapered oesophageal narrowing is infradiaphragmatic in location and partial intermittent relaxation is often demonstrated fluoroscopically. REFERENCES
AREY, L. B., 1954. Developmental Anatomy, 6th edn.
(Saunders, Philadelphia).
ASTLEY, R., 1956. Radiology of the Alimentary Tract in Infancy (Edward Arnold, Ltd., London). ASTLEY, R., and CARRE, I. J., 1954. Gastroesophageal
incompetence in children with special reference to minor degrees of partial thoracic stomach. Radiology, 62, 351— 361. BERGMANN, M., and CHARNAS, R. H., 1958. Tracheo-
bronchial rests in the esophagus. Journal of Thoracic Surgery, 35, 97-104. CASTLEMAN, B., 1956. Case 42411. Case records of Massachusetts General Hospital. New England Journal of Medicine, 255, 707-710. CAFFEY, J., 1972. Pediatric X-ray Diagnosis. 6th edn., pp.596-597 (Year Book Publishers, Chicago). DUNBAR, J. S., 1958. Congenital oesophageal stenosis. Pediatric Clinics of North America, 5, 443-455. FREY, E. K., and DUSCHL, L., 1936. Der Kardiospasmus.
Ergebnisse der Chirurgie und Orthopddie, 29, 637-716. GENIESER, N. B., and BECKER, M. H., 1969. "Clinitest
strictures" of the esophagus. Clinical Pediatrics, 8, {Supplement) 17A-19A. ISHIDA, M., TSUCHIDA, Y., SAITO, S., and TSUNODA, A.,
1969. Congenital esophageal stenosis due to tracheobronchial remnants. Journal of Pediatric Surgery, 4, 339-345. KUMAR, R., 1962. A case of congenital esophageal stricture due to a cartilaginous ring. British Journal of Surgery, 49, 533-534. MAGILNER, A. D., and ISARD, H. J., 1971. Achalasia of the
esophagus in infancy. Radiology, 98, 81-82. MUSTARD, W. T., RAVITCH, M. M., SNYDER, W. H., WELCH, K. J., and BENSON, C. D., 1969. Pediatric Sur-
gery, 2nd edn. (Year Book Publishers, Chicago). PAULINO, F., ROSELLI, A., and APRIGLIANO, F., 1963.
18
Congenital esophageal stricture due to tracheobronchial remnants. Surgery, 53, 547—550. SPATH, VON F., and RATZENHOFER, M., 1959. Ueber die
angeborene Oesophagusstenose. Wiener Klinische Wochenschrift, 71, 723-727.
Congenital oesophageal strictures due to cartilaginous rings By Judith S. Rose, M.D., E. G. Kassner, M.D., K. H. Jurgens, M.D., and J. Farman, M.B., Ch.B., M.Med. Departments of Radiology and Pathology, Downstate Medical Center, Brooklyn, New York (Received December, 1973)
and fever. Past history included multiple bouts of vomiting and recurrent respiratory infections since birth. Multiple features of trisomy 21, including microcephaly, mongoloid facies, bilateral cataracts, and retarded mental and physical development were present. A barium swallow disclosed oesophageal dilatation proximal to a 4 cm narrowing of the distal oesophagus adjacent to the cardia. Projecting from the medial wall of the midportion of this stricture was a 0-5 cm collection of barium suggestive of an ulceration (Fig. 3). A firm unyielding stenosis was encountered 31 cm below the incisors at oesophagoscopy. The patient was referred for surgery and the stenosis was excised. The patient graduated from a liquid to semi-solid diet without difficulty. She was, however, found dead in bed two weeks post-operatively and the cause of death was ascribed to aspiration.
ABSTRACT
Two cases of distal oesophageal stricture secondary to tracheo-bronchial remnants containing cartilage are presented. The clinical histories and radiographic appearances of these lesions are discussed as is the postulated embryogenesis. A characteristic finding, when present, is the linear collection of barium projecting horizontally from the area of stricture, representing respiratory epithelial ducts. Tracheobronchial cartilaginous rests are rarely found in the distal oesophagus, but when present may be responsible for congenital narrowing. This anomaly is usually encountered in the neonate or young infant when symptoms of dysphagia, vomiting and aspiration pneumonia occur (Kumar, 1962; Paulino, Roselli and Aprigliano, 1963). Sometimes symptoms may be unrecognized in the younger patient; a number of cases have been reported in adults (Bergmann and Charnas, 1958; Castleman, 1956; Frey and Duschl, 1936; Spath and Ratzenhofer, 1959). CASE REPORTS
Case 1 M.B., a seven-month old infant was admitted to Kings County Hospital in July 1971 with a two-day history of difficulty in swallowing. A barium study demonstrated dilatation of the oesophagus with persistent narrowing of the distal segment measuring 2 cm in length just proximal to the oesophagogastric junction (Fig. 1). At oesophagoscopy, several pieces of cardboard were wedged in the distal oesophagus and were removed. Four months later, the infant was readmitted with a sixday history of dysphagia and regurgitation of both liquids and solids. Several pieces of newspaper were present in the pharynx and these were removed manually. Nasogastric feedings were instituted and a repeat oesophagram disclosed that the radiographic appearances were unchanged. The patient was referred for surgery and the distal stenosed oesophagus was excised and an end-to-end anastomosis effected. The post-operative course was uneventful and oesophagram revealed a satisfactory patent anastomosis. Macroscopic description of specimen The resected strictured segment showed a number of firm, grey bands running circumferentially in the thickened wall. Microscopic description of specimen The lumen was lined by stratified squamous epithelium. The submucosa and muscularis were not well organized and contained mucus glands with ducts connecting with the oesophageal lumen. Several cartilage bands were located in the muscularis; the largest extended for nearly half the circumference of the wall. Ductlike structures with pseudostratified columnar epithelium, surrounded by dense aggregates of lymphocytes, were located both internal and external to the cartilage rings (Fig. 2). Case 2 L.H., an eight-year old female was first admitted to Kings County Hospital with a two-day history of cough 16
FIG. 1. Case 1. Barium swallow demonstrating the lower oesophageal stricture with dilatation above.
JANUARY
1975
Congenital oesophageal strictures due to cartilaginous rings
FIG. 2. Photomicrography showing a cross section of the oesophagus. (H&ExlO) L = Lumen C = Cartilage bands G = Glands D = Ducts E = Pseudostratified columnar epithelium surrounded by lymphoid aggregates.
Macroscopic description of specimen
A 3 X 2-5 X 0-5 cm segment of oesophagus was examined. It was bony hard in consistency and the mucosal surface had a pinkish-grey colour. There was no evidence of ulceration, but a 0 5 cm tract was present and extended from the medial aspect of the stenosed segment. Microscopic description of specimen
The oesophageal lumen was lined by stratified squamous mucosa and there was a disorganized submucosa and muscularis which contained numerous secreting glands and scattered mixed glands. Several cartilage plates and ductlike structures lined by ciliated pseudostratified columnar epithelium were present. DISCUSSION
Congenital oesophageal stenosis due to tracheobronchial remnants was first described in a 19-year17
FIG. 3. Case 2. Frontal view of a barium swallow demonstrating a markedly dilated oesophagus with a stenosed distal segment. There is a 0-5 cm tract extending medially from the strictured portion of the oesophagus.
old female by Frey and Duschl. The second case reported was also in an adult, a patient who was 52 years old with a history of intermittent dysphagia since the age of three (Castleman, 1956). While several cases in adults have been reported (Bergmann and Charnas, 1958; Spath and Ratzenhofer, 1959) the majority of patients are children whose ages range from eight months to five years when first diagnosed (Ishida et al., 1969). No sex predilection exists (Ishida et al., 1969). These anomalies have been presumed to result from a failure of normal embryonic separation of the respiratory tract from the primitive oesophagus. Other developmental disorders with tracheobronchial remnants probably belong to this category:
VOL.
48, No. 565 Judith S. Rose, E. G. Kassner, K. H. Jurgens and J. Farman
(1) tracheoesophageal fistula; (2) cysts lined by bronchial epithelium, whether mediastinal, pulmonary or intramurally situated in the oesophagus; and (3) diverticula of the oesophagus containing tracheobronchial tissue (Bergmann and Charnas, 1958; Ishida «**/., 1969). The tracheobronchial groove, the primordium of the respiratory system develops as a median ventral diverticulum of the foregut. This groove is intimately related to the oesophagus which develops from the foregut caudal to the respiratory diverticulum. Lateral indenting grooves form on both sides, eventually separating the respiratory primordium from the oesophagus (Arey, 1954). Kumar (1962) and Paulino et al. (1963), have hypothesized that tracheal cartilage sequestrates in the oesophagus before the two tubes have completely separated from each other. Since this cartilage is usually found in the distal oesophagus, these cells may well have been carried down the oesophagus during the normal process of growth. Many of the reported cases of congenital stenosis of the oesophagus have both cartilage and mucous glands in the stenotic segment (Ishida et al., 1969). Respiratory-like epithelium has commonly been identified in this abnormality (Castleman, 1956; Paulino et al., 1963) and it may be surrounded by lymphoid tissue (Bergmann and Charnas, 1958; Ishida et al., 1969). The origin of these lymphoid structures is unexplained. The radiographic appearance in our two patients was indicative of a non-neoplastic stricture of the distal oesophagus with secondary proximal dilatation. In our second case, there was a tiny collection of barium projecting medially from the midportion of the oesophageal narrowing. This was an important radiological finding in the evaluation of the stricture. Similar appearances have been described in the literature (Castleman, 1956; Kumar, 1962; Spath and Ratzenhofer, 1959). The significance of this finding, however, was not fully appreciated and at times was wrongly attributed to ulceration within the stricture. Pathologically, these barium-filled projections have proved to be ducts communicating with the oesophageal lumen. Multiple bilateral projections have been observed (Castleman, 1956; Spath and Ratzenhofer, 1959). The differential diagnosis of these congenital strictures includes stenoses secondary to ingestion of caustic agents, peptic oesophagitis with or without hiatus hernia, and less commonly achalasia. Caustic agents such as lye (Caffey, 1972) or
clinitest tablets (Genieser and Becker, 1969) can damage the oesophageal mucosa resulting in fibrosis and stricture. Corrosive strictures tend to be long in configuration and the history and initial endoscopic findings are diagnostic. Ingestion of clinitest tablets produces a circumferential narrowing, most often seen at the level of the aortic knuckle (Genieser and Becker, 1969). Reflux oesophagitis is frequently associated with hiatus hernia and is another cause of oesophageal stricture (Astley, 1956; Astley and Carre, 1954; Mustard et al., 1969). Ulcerations progress to fibrosis and tight stricture formation. In most instances the differential diagnosis is made by correlation of the history, endoscopic findings and early radiographic appearance of this lesion. Achalasia in the paediatric population is rare, especially in patients under one year of age (Magilner and Isard, 1971). The tapered oesophageal narrowing is infradiaphragmatic in location and partial intermittent relaxation is often demonstrated fluoroscopically. REFERENCES
AREY, L. B., 1954. Developmental Anatomy, 6th edn.
(Saunders, Philadelphia).
ASTLEY, R., 1956. Radiology of the Alimentary Tract in Infancy (Edward Arnold, Ltd., London). ASTLEY, R., and CARRE, I. J., 1954. Gastroesophageal
incompetence in children with special reference to minor degrees of partial thoracic stomach. Radiology, 62, 351— 361. BERGMANN, M., and CHARNAS, R. H., 1958. Tracheo-
bronchial rests in the esophagus. Journal of Thoracic Surgery, 35, 97-104. CASTLEMAN, B., 1956. Case 42411. Case records of Massachusetts General Hospital. New England Journal of Medicine, 255, 707-710. CAFFEY, J., 1972. Pediatric X-ray Diagnosis. 6th edn., pp.596-597 (Year Book Publishers, Chicago). DUNBAR, J. S., 1958. Congenital oesophageal stenosis. Pediatric Clinics of North America, 5, 443-455. FREY, E. K., and DUSCHL, L., 1936. Der Kardiospasmus.
Ergebnisse der Chirurgie und Orthopddie, 29, 637-716. GENIESER, N. B., and BECKER, M. H., 1969. "Clinitest
strictures" of the esophagus. Clinical Pediatrics, 8, {Supplement) 17A-19A. ISHIDA, M., TSUCHIDA, Y., SAITO, S., and TSUNODA, A.,
1969. Congenital esophageal stenosis due to tracheobronchial remnants. Journal of Pediatric Surgery, 4, 339-345. KUMAR, R., 1962. A case of congenital esophageal stricture due to a cartilaginous ring. British Journal of Surgery, 49, 533-534. MAGILNER, A. D., and ISARD, H. J., 1971. Achalasia of the
esophagus in infancy. Radiology, 98, 81-82. MUSTARD, W. T., RAVITCH, M. M., SNYDER, W. H., WELCH, K. J., and BENSON, C. D., 1969. Pediatric Sur-
gery, 2nd edn. (Year Book Publishers, Chicago). PAULINO, F., ROSELLI, A., and APRIGLIANO, F., 1963.
18
Congenital esophageal stricture due to tracheobronchial remnants. Surgery, 53, 547—550. SPATH, VON F., and RATZENHOFER, M., 1959. Ueber die
angeborene Oesophagusstenose. Wiener Klinische Wochenschrift, 71, 723-727.
