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Longitudinal esophageal bands associated with esophageal aperistalsis
00165065/78/7493-0592$02.09/0 G--WY 7459%594.1978 Copyright Q l978by theAmerican Ga.stroentemlogical Association
Vol. 74, No. 3 Printed in USA.
LONGITUDINAL ESOPHAGEAL BANDS ASSOCIATED WITH ESOPHAGEAL APERISTALSIS Speculations on pathogenesis ALFRED E. STILLMAN, M.D.,
WILLIAM LARTER, M.D.,
AND DAVID S. GOLDMAN, M.D.
Departments of Gastroentemlogy and Pathology, Tutison Hospitals Medical Education Program, Tucson Medical Center, and the Department ofPediatrics, University ofArizona College of Medicine
A B-year-old girl with ataxia telangiectasia syndrome was found to have multiple bands longitudinally traversing her esophagus, and esophageal aperistalsis. In the past, the patient was also known to have had esophageal candidiasis. The bands may have been congenital or may have been produced when linear ulcers caused by fungal esophagitis undermined mucosal strips and then healed, leaving tissue bands attached only at their extremities to the esophageal wall. The esophageal motor dysfunction may have been attributable to esophageal candidiasis, or may have been a hitherto unrecognized component of the ataxia telangiectasia syndrome. Although esophageal webs are well known,‘~ 2 longitudinal esophageal bands have not been described. These bands occurred in a young girl with dysphagia, ataxia telangiectasia syndrome, and esophageal candidiasis. The patient was also found to have esophageal aperistalsis that antedated the clinical dysphagia. Speculations on the pathogenesis of the esophageal bands and motor dysfunction form the basis of this report.
either to immrmodeficiency or to aspiration. Four months before admission, the patient developed postprandial emesis without change in diet. Physical examination revealed oral thrush, although this was not confirmed by either smear or culture. A barium esophagogram was not performed. She was empirically treated with 400,600 units of nystatin in an aqueous vehicle four times a day for 2 weeks. Both oral plaques and emesis resolved on this therapy, During the 2 years before admission, the patient had not received oral medications other than liquid nystatin and, just before admisCase Report sion, levamisole, a drug that may augment deficient cellA B-year-old girl with ataxia telangiectasia syndrome was mediated immunity.3 Shortly after admission, the patient underwent upper gasadmitted to Tucson Medical Center for evaluation of probable trointestinal endoscopy using the Olympus GIFP2 (pediatric) dysphagia for solid foods during the preceding 16 months. The patient was known to have esophageal aperistalsis for at instrument. The proximal esophagus was normal. However, least 2 years before admission. Esophagoscopy had been per- the distal esophagus was longitudinally traversed by four formed 1 year before the present admission to investigate the bands that were firmly attached to the esophageal wall near abnormal esophageal motility. At that time, the patient the midesophagus and the gastroesophageal junction (fig. 1, complained of high epigastric discomfort and frequent emesis A and B). Manipulation of these bands with a biopsy forceps revealed that they were free of attachment to the esophagus after ingestion of solid food; the discomfort lasted for 2 hr after meals. Examination with the Olympus fiberoptic bron- except at their extreme ends. Biopsies of these bands revealed an internal fibrous structure covered by epithelium exhibiting choscope (model BFdB2, Olympus Corporation of America, New Hyde Park, N. Y.) revealed moderately severe distal the histological characteristics of chronic esophagitip (fig. 2); esophagitis confirmed by esophageal biopsies; no other gross special stains failed to reveal evidence of fungi. The gastroesophageal junction was competent; the stomach, duodenal or histological esophageal abnormality was noted then. Howbulb, and first and second portions of the duodenum appeared ever, poor visualization, owing to the small suction port and normal. On the following day, a barium cine-esophagogram inability to insufflate air easily was noted throughout the using both standard and thick barium and air contrast was procedure. Following that examination, the patient was performed by a radiologist who had been apprised of the treated with elevation of the head of her bed, postprandial endoscopic findings; again, aperistalsis was documented but antacids, and an elemental diet (Vivonex, Eaton Laboratories, neither reflux nor the bands were seen. Esophageal manomeNorwich, N. Y.). The epigastric discomfort disappeared but try revealed a lower esophageal sphincter pressure of 16 mm reappeared upon attempted reintroduction of solid food into the diet. Elevation of the head of the bed and antacids as Hg, normal for this patient’s age.5 The patient was discharged with continued instruction to the parents to maintain elevaneeded were maintained until the present admission. During the 2 years before admission, the patient had three tion of the head of the bed and to blender-ire her food, to episodes of pneumonia; their etiology may have been related which she responded with increased oral intake and slow but persistent weight gain. ReceivedJuly 11,19’77.AcceptedOctober18, 1977. During investigation into the cause of the esophageal Addressrequestsfor reprintsto: Alfred E. Stillman, M.D., 5200 bands, the esophageal biopsies obtained 1 year previously East Grant Road, Suite 611, Tucson,Arizona 85712. were reviewed; hyphae morphologically resembling a Candida 592
CASE
March 1978
Band,
B
593
REPORTS
Band
G-E Junction /
FIG. 1. A, endoscopic view of esophagus just proximal to the gastroesophageal junction. Three bands are clearly seen; they cast prominent shadows due to their separation from the esophageal wall. B, artistic representation of the endoscopic photograph and a longitudinal section through the patient’s esophagus. The line through the distal esophagus is the level at which the photograph was taken.
esophagogl am was then performed, but esophageal aperistalsis persisted unchanged and reflux was not seen.
Discussion
FIG. 2. Biopsy of a band revealing an internal fibrous structure and squamous mucosa with a prominent basal layer and rem pegs approaching the luminal surface. species were found. Although no evidence of fungal infection was found during the second endoscopic examination, the patient was treated with 200,000 units of nystatin dissolved in 2 ml of methylcellulose every hour while awake for 1 week, and four times a day for 4 additional weeks.6 A barium cine-
The pathogenesis of the esophageal bands in this patient is difficult to determine. If the bands were acquired, esophageal candidiasis may have been responsible for their origin. Linear monilial esophageal ulcerations may have undermined thin strips of inflamed mucosa, and possibly submucosa, eventually freeing those strips from all attachment to the esophageal wall except at their most proximal and distal ends. Healing of the monilial ulcers, perhaps in response to nystatin, may then have produced the appearance encountered at the second endoscopic examination. Had the undermining process taken place gradually, neovascularization of the entire band might have occurred from capillary buds at either end of the band. With continued fibroblastic proliferation and collagen deposition associated with healing, the band would have been eventually transformed into a relatively avascular structure with a fibrous core and an epithelial surface layer.’ Alternatively, the presence of four bands originating from and inserting into the same level of the esophagus suggests a congenital origin. It seems unlikely that four such bands would have been undermined by eight linear monilial ulcerations of similar length and depth. The changes of chronic esophagitis in the mucosa covering the bands are consistent with either an acquired or congenital origin. The etiology of the patient’s esophageal aperistalsis, documented on three tine-esophagogram studies over 2 years, is likewise speculative. In achalasia, such motor dysfunction may be asymptomatic for a long time before
594
Vol.74,No.3
CASE REPORTS
resulting in dysphagia for solid food, one of the earliest symptoms of this disorder in childhood.8*s This patient probably did not have achalasia because her lower esophageal sphincter pressure was normal rather than elevated.8 The presence of gastroesophageal reflux, another possible cause of esophageal aperistalsis,1° was never conclusively proved. The initial apparent symptomatic response to antacids and positional therapy was probably attributable only to gravity-induced drainage of the esophagus. Aperistalsis may have been secondary to intense monilial esophagitis.” The ineffectiveness of nystatin in restoring esophageal peristalsis indicates the lack of association between the motor disorder and active monilial infection in this patient. However, subtle fibrotic changes in the esophageal submucosal or muscular layers similar to the changes observed in the bands may have altered motility, even though the esophageal walls appeared normally pliable and distensible during both radiological and endoscopic examination. Finally, the motor disorder may have been an integral part of the ataxia telangiectasia syndrome. Although an association between esophageal peristaltic abnormalities and ataxia telangiectasia syndrome has not been previously noted, such an association may nevertheless exist. Esophageal cine-esophagograms or manometric studies of patients with this syndrome followed in appropriate cases by esophagoscopy may disclose clinically unsuspected esophageal motor disorders unrelated to monilial esophagitis. Aperistalsis in our patient may have been asymptomatic; dysphagia either caused by or aggravated by the bands
may have directed attention to the esophagus with subsequent investigation of the motor disorder and discovery of the bands. REFERENCES Chisholm M, Ardran G, Callender S, et al: Iron deficiency and autoimmunity in post-cricoid webs. Q J Med 40~421-433,1971 Tedesco F, Morton W: Lower esophageal webs. Am J Dig Dis 20:381-383, 1975 Lieberman R, Hsu M: Levamisole-mediated restoration of cellular immunity in peripheral blood lymphocytes of patients with immunodeficiency diseases. Clin Immunol Immunopathol5:142146,1976 4.Ismail-Beigi F, Horton P, Pope C: Histological consequences of gastroesophageal reflux in man. Gastroenterology 58163-174, 1970 5.Euler A, Fonkalsrud E, Ament M: Effect of Nissen fundoplication on the lower esophageal sphincter pressure of children with gastroesophageal reflux. Gastroenterology 72:260-262,1977 6.Kantrowitz P, Fleishli D, Butler W: Successful treatment of chronic esophageal moniliasis with a viscous suspension of nystatin. Gastroenterology 57:424-430, 1969 7.Robbin S: Pathologic Basis of Disease. First edition. Philadelphia, WB Saunders Co, 1974, p 92-93 8.Roy C, Silverman A, Cozzetto F: Pediatric Clinical Gastroenterology, Second edition. St. Louis, CV Mosby Co, 1975, p 148-152 9.Gryboski J: Gastrointestinal Problems in the Infant. First edition. Philadelphia, WB Saunders Co, 1975, p 87-92 10. Donner M, Silbinger M, Hookman P, et al: Acid-barium swallows in the radiographic evaluation of clinical esophagitis. Radiology 87:220-225, 1966 11. Mukhopadhyay A, Graham D: Esophageal motor dysfunction in systemic diseases. Arch Intern Med 136:583-588, 1976
Vol. 74, No. 3 Printed in USA.
LONGITUDINAL ESOPHAGEAL BANDS ASSOCIATED WITH ESOPHAGEAL APERISTALSIS Speculations on pathogenesis ALFRED E. STILLMAN, M.D.,
WILLIAM LARTER, M.D.,
AND DAVID S. GOLDMAN, M.D.
Departments of Gastroentemlogy and Pathology, Tutison Hospitals Medical Education Program, Tucson Medical Center, and the Department ofPediatrics, University ofArizona College of Medicine
A B-year-old girl with ataxia telangiectasia syndrome was found to have multiple bands longitudinally traversing her esophagus, and esophageal aperistalsis. In the past, the patient was also known to have had esophageal candidiasis. The bands may have been congenital or may have been produced when linear ulcers caused by fungal esophagitis undermined mucosal strips and then healed, leaving tissue bands attached only at their extremities to the esophageal wall. The esophageal motor dysfunction may have been attributable to esophageal candidiasis, or may have been a hitherto unrecognized component of the ataxia telangiectasia syndrome. Although esophageal webs are well known,‘~ 2 longitudinal esophageal bands have not been described. These bands occurred in a young girl with dysphagia, ataxia telangiectasia syndrome, and esophageal candidiasis. The patient was also found to have esophageal aperistalsis that antedated the clinical dysphagia. Speculations on the pathogenesis of the esophageal bands and motor dysfunction form the basis of this report.
either to immrmodeficiency or to aspiration. Four months before admission, the patient developed postprandial emesis without change in diet. Physical examination revealed oral thrush, although this was not confirmed by either smear or culture. A barium esophagogram was not performed. She was empirically treated with 400,600 units of nystatin in an aqueous vehicle four times a day for 2 weeks. Both oral plaques and emesis resolved on this therapy, During the 2 years before admission, the patient had not received oral medications other than liquid nystatin and, just before admisCase Report sion, levamisole, a drug that may augment deficient cellA B-year-old girl with ataxia telangiectasia syndrome was mediated immunity.3 Shortly after admission, the patient underwent upper gasadmitted to Tucson Medical Center for evaluation of probable trointestinal endoscopy using the Olympus GIFP2 (pediatric) dysphagia for solid foods during the preceding 16 months. The patient was known to have esophageal aperistalsis for at instrument. The proximal esophagus was normal. However, least 2 years before admission. Esophagoscopy had been per- the distal esophagus was longitudinally traversed by four formed 1 year before the present admission to investigate the bands that were firmly attached to the esophageal wall near abnormal esophageal motility. At that time, the patient the midesophagus and the gastroesophageal junction (fig. 1, complained of high epigastric discomfort and frequent emesis A and B). Manipulation of these bands with a biopsy forceps revealed that they were free of attachment to the esophagus after ingestion of solid food; the discomfort lasted for 2 hr after meals. Examination with the Olympus fiberoptic bron- except at their extreme ends. Biopsies of these bands revealed an internal fibrous structure covered by epithelium exhibiting choscope (model BFdB2, Olympus Corporation of America, New Hyde Park, N. Y.) revealed moderately severe distal the histological characteristics of chronic esophagitip (fig. 2); esophagitis confirmed by esophageal biopsies; no other gross special stains failed to reveal evidence of fungi. The gastroesophageal junction was competent; the stomach, duodenal or histological esophageal abnormality was noted then. Howbulb, and first and second portions of the duodenum appeared ever, poor visualization, owing to the small suction port and normal. On the following day, a barium cine-esophagogram inability to insufflate air easily was noted throughout the using both standard and thick barium and air contrast was procedure. Following that examination, the patient was performed by a radiologist who had been apprised of the treated with elevation of the head of her bed, postprandial endoscopic findings; again, aperistalsis was documented but antacids, and an elemental diet (Vivonex, Eaton Laboratories, neither reflux nor the bands were seen. Esophageal manomeNorwich, N. Y.). The epigastric discomfort disappeared but try revealed a lower esophageal sphincter pressure of 16 mm reappeared upon attempted reintroduction of solid food into the diet. Elevation of the head of the bed and antacids as Hg, normal for this patient’s age.5 The patient was discharged with continued instruction to the parents to maintain elevaneeded were maintained until the present admission. During the 2 years before admission, the patient had three tion of the head of the bed and to blender-ire her food, to episodes of pneumonia; their etiology may have been related which she responded with increased oral intake and slow but persistent weight gain. ReceivedJuly 11,19’77.AcceptedOctober18, 1977. During investigation into the cause of the esophageal Addressrequestsfor reprintsto: Alfred E. Stillman, M.D., 5200 bands, the esophageal biopsies obtained 1 year previously East Grant Road, Suite 611, Tucson,Arizona 85712. were reviewed; hyphae morphologically resembling a Candida 592
CASE
March 1978
Band,
B
593
REPORTS
Band
G-E Junction /
FIG. 1. A, endoscopic view of esophagus just proximal to the gastroesophageal junction. Three bands are clearly seen; they cast prominent shadows due to their separation from the esophageal wall. B, artistic representation of the endoscopic photograph and a longitudinal section through the patient’s esophagus. The line through the distal esophagus is the level at which the photograph was taken.
esophagogl am was then performed, but esophageal aperistalsis persisted unchanged and reflux was not seen.
Discussion
FIG. 2. Biopsy of a band revealing an internal fibrous structure and squamous mucosa with a prominent basal layer and rem pegs approaching the luminal surface. species were found. Although no evidence of fungal infection was found during the second endoscopic examination, the patient was treated with 200,000 units of nystatin dissolved in 2 ml of methylcellulose every hour while awake for 1 week, and four times a day for 4 additional weeks.6 A barium cine-
The pathogenesis of the esophageal bands in this patient is difficult to determine. If the bands were acquired, esophageal candidiasis may have been responsible for their origin. Linear monilial esophageal ulcerations may have undermined thin strips of inflamed mucosa, and possibly submucosa, eventually freeing those strips from all attachment to the esophageal wall except at their most proximal and distal ends. Healing of the monilial ulcers, perhaps in response to nystatin, may then have produced the appearance encountered at the second endoscopic examination. Had the undermining process taken place gradually, neovascularization of the entire band might have occurred from capillary buds at either end of the band. With continued fibroblastic proliferation and collagen deposition associated with healing, the band would have been eventually transformed into a relatively avascular structure with a fibrous core and an epithelial surface layer.’ Alternatively, the presence of four bands originating from and inserting into the same level of the esophagus suggests a congenital origin. It seems unlikely that four such bands would have been undermined by eight linear monilial ulcerations of similar length and depth. The changes of chronic esophagitis in the mucosa covering the bands are consistent with either an acquired or congenital origin. The etiology of the patient’s esophageal aperistalsis, documented on three tine-esophagogram studies over 2 years, is likewise speculative. In achalasia, such motor dysfunction may be asymptomatic for a long time before
594
Vol.74,No.3
CASE REPORTS
resulting in dysphagia for solid food, one of the earliest symptoms of this disorder in childhood.8*s This patient probably did not have achalasia because her lower esophageal sphincter pressure was normal rather than elevated.8 The presence of gastroesophageal reflux, another possible cause of esophageal aperistalsis,1° was never conclusively proved. The initial apparent symptomatic response to antacids and positional therapy was probably attributable only to gravity-induced drainage of the esophagus. Aperistalsis may have been secondary to intense monilial esophagitis.” The ineffectiveness of nystatin in restoring esophageal peristalsis indicates the lack of association between the motor disorder and active monilial infection in this patient. However, subtle fibrotic changes in the esophageal submucosal or muscular layers similar to the changes observed in the bands may have altered motility, even though the esophageal walls appeared normally pliable and distensible during both radiological and endoscopic examination. Finally, the motor disorder may have been an integral part of the ataxia telangiectasia syndrome. Although an association between esophageal peristaltic abnormalities and ataxia telangiectasia syndrome has not been previously noted, such an association may nevertheless exist. Esophageal cine-esophagograms or manometric studies of patients with this syndrome followed in appropriate cases by esophagoscopy may disclose clinically unsuspected esophageal motor disorders unrelated to monilial esophagitis. Aperistalsis in our patient may have been asymptomatic; dysphagia either caused by or aggravated by the bands
may have directed attention to the esophagus with subsequent investigation of the motor disorder and discovery of the bands. REFERENCES Chisholm M, Ardran G, Callender S, et al: Iron deficiency and autoimmunity in post-cricoid webs. Q J Med 40~421-433,1971 Tedesco F, Morton W: Lower esophageal webs. Am J Dig Dis 20:381-383, 1975 Lieberman R, Hsu M: Levamisole-mediated restoration of cellular immunity in peripheral blood lymphocytes of patients with immunodeficiency diseases. Clin Immunol Immunopathol5:142146,1976 4.Ismail-Beigi F, Horton P, Pope C: Histological consequences of gastroesophageal reflux in man. Gastroenterology 58163-174, 1970 5.Euler A, Fonkalsrud E, Ament M: Effect of Nissen fundoplication on the lower esophageal sphincter pressure of children with gastroesophageal reflux. Gastroenterology 72:260-262,1977 6.Kantrowitz P, Fleishli D, Butler W: Successful treatment of chronic esophageal moniliasis with a viscous suspension of nystatin. Gastroenterology 57:424-430, 1969 7.Robbin S: Pathologic Basis of Disease. First edition. Philadelphia, WB Saunders Co, 1974, p 92-93 8.Roy C, Silverman A, Cozzetto F: Pediatric Clinical Gastroenterology, Second edition. St. Louis, CV Mosby Co, 1975, p 148-152 9.Gryboski J: Gastrointestinal Problems in the Infant. First edition. Philadelphia, WB Saunders Co, 1975, p 87-92 10. Donner M, Silbinger M, Hookman P, et al: Acid-barium swallows in the radiographic evaluation of clinical esophagitis. Radiology 87:220-225, 1966 11. Mukhopadhyay A, Graham D: Esophageal motor dysfunction in systemic diseases. Arch Intern Med 136:583-588, 1976