CASE REPORTS. Giant Esophagus. An Unusual Case of Massive Idiopathic Hypertrophy

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1 CASE REPORTS An Unusual Case of Massive Idiopathic Hypertrophy and Dilatation of the Esophagus and Proximal Stomach Mark H. Wall, M.D., Epifanio E. Espinas, M.D., Arthur W. Silver, M.D., and Francis X. Byron, M.D. T here are only a few conditions described which are associated with muscular hypertrophy or dilatation of the esophagus. Among these conditions are achalasia, idiopathic diffuse muscular hypertrophy of the lower esophagus [9], and diffuse spasm of the esophagus [6]. It is interesting that hypertrophy and dilatation generally do not occiir in response to obstructions due to benign strictures or malignant neoplasms but are seen in the so-called functional group of disorders. A very unusual case of giant esophagus treated at the City of Hope merits discussion because it presented unusual clinical and anatomical features not. generally described in the conditions mentioned above and because of ithe difficult problems in surgical management it presented and the rather radical surgical treatment it required. G. H., a 29-year-old white housewife, was admitted for the first time on September 23, 1965, with the following history: In March, 1965, a routine chest film had been taken when the patient was in good health and denied any serious gastrointestinal symptoms; it showed an abnormality. Further studies suggested the presence of a tumor in the distal portion of the esophagus (Fig. 1). In June, 1965, several hundred grams of what was reported to be leiomyoma of the esophagus was remcted through the left chest. The surgeon reported that this tumor was too large to be completely removed through a left thoracotomy and that it extended far into the right chest. The patient had a stormy postoperative course From the City of Hope Medical Center, Duarte, Calif. Accepted for publication Jan. 23, THE ANNALS OF THORACIC SURGERY

CASE REPORT: A B FIG. 1. (A) Plain x-ray prior to first operation shows deviation of thick-walled esophagzis into the right chest. (B, C) An esophagram shows barium mixed with retained food.

One month later, in July, 1965, the patient was again hospitalized.

2 CASE REPORT: A B FIG. 1. (A) Plain x-ray prior to first operation shows deviation of thick-walled esophagzis into the right chest. (B, C) An esophagram shows barium mixed with retained food. The cardioesophageal junction tapers as in achalasia; (C) shows the hypertrophy of the wall outlined against the column of barium. and required numerous blood transfusions and a tracheostomy. One month later, in July, 1965, the patient was again hospitalized. A laparotomy and a right thoracotomy were done with the intent of resecting the esophageal tumor and performing a thoracic esophagogastrostomy. Again the surgeon reported the lesion was too extensive to resect. A stormy febrile postoperative course followed, and again a tracheostomy and multiple transfusions were required. The patient was discharged for a short time, but because of continued fever and a fall in hemoglobin to 3 gm. per 100 cc. rehospitalization was required in late September, The patient s weight had dropped from 125 to 89 pounds, and she had received approximately 25 blood transfusions. At this point the patient was transferred to the City of Hope Medical Center. On admission physical examination revealed a tall, thin, febrile, acutely ill woman showing evidence of recent marked weight loss. Her mucous membranes were pale, and her pulse was rapid. A recent tracheostomy incision was unhealed. Healing posterolateral thoracotomy incisions were present on both right and left chest, and a recent vertical midline incision was present on the upper abdomen. C VOL. 4, NO. I, JULY,

WALL, ESF INAS, SILVER, AND BYRON FIG. 2. An anteioposterior view of the esophagus at the time of admission to City of Hopc demonstrates the massive thickening and dilatation of the wall.

3 WALL, ESF INAS, SILVER, AND BYRON FIG. 2. An anteioposterior view of the esophagus at the time of admission to City of Hopc demonstrates the massive thickening and dilatation of the wall. Past history revealed that a hysterectomy was done two years earlier for an unusually large fibroid uterus, that a diagnosis of bronchitis had been made in the past, that the patient had complained of excessive mucous in her throat for several years, and that she had frequently been treated for anemia. Laboratory Data. Hemoglobin was 10.3 gm. per 100 cc., and total proteins were 5.8, albumin 3, and globulin 2.8 gm. per 100 cc. X-rays. Chest x-rays and a barium swallow showed a greatly dilated, tortuous esophagus with massive thickening of its wall and wide deviation into the right hemithorax (Fig. 2). The patient was too ill for more complete x-ray examination at this time. For this reason and because of retention of and very slow emptying of barium from the esophagus, the esophagogastric junction and the proximal stomach could not be evaluated well. Hospital Course. At this point the exact nature of the lesion was uncertain. The referral diagnosis was leiomyoma oe the esophagus; but in any event, it was apparent that serious complications had occurred. Because the continuing blood loss and spiking fever were an immediate threat to the patient s life, exploration of the right chest was recommended for resection of the esophageal lesion if possible. A right thoracotomy was carried out on October 1, At operation a giant, hypertrophied, tortuous esophagus was found. It measured 10 cm. in diameter at its distal end and 6 cm. in external diameter at the level of the azygos vein. The distal muscular wall of the esophagus measured 3 cm. in thickness. Proximally the wall gradually thinned and the diameter of the esophagus decreased until the organ reached normal wall thickness and external diameter at the level of the first chondrosternal junction. An abscess cavity was entered and drained in the region of the esophageal hiatus. As the dissection progressed, an esophageal hiatus hernia of approximately 20% to 30% of the stomach was encountered. The wall of the visualized stomach was like the esophagus-markedly hypertrophic. Within the thoracic portion of the stomach, a giant gastric ulcer crater measuring 6 X 4 cm. and penetrating the full thickness of the gastric wall was found. The thoracic aorta formed the floor of the ulcer. Esophagitis and several shallow ulcerations were seen on the distal esophageal mucosa. The cardioesophageal junction was unobstructed and actually had a lumen larger than normal. The mas:,ive thoracic esophagus was resected along with a portion of the hypertrophied proximal stomach including the gastric ulcer (Fig. 3). It was not possible to place the stomach below the diaphragm from the right chest approach. 62 THE ANNALS OF THORACIC SURGERY

A cervical esophagostomy was placed on the left neck, and a tube gastrostomy completed the operation. The initial postoperative course was smooth, then fever and leukocytosis developed.

4 CASE REPORT: FIG. 3. Iisophageal portion of the resected specimen. Note the thickness of the esophageal wall (?,O cm.) at the esophagogastric junction in the left one-third of the specimen. The? cm. starting at the left of the specimen is the gastric wall, also massively hypertrophied. A cervical esophagostomy was placed on the left neck, and a tube gastrostomy completed the operation. The initial postoperative course was smooth, then fever and leukocytosis developed. X-ray studies showed leakage of contrast media from the supradiaphragmatic gastric pouch. On October 13, 1965, a left thoracotomy was carried out. The abnormally thickened leaking gastric pouch was resected, closed, and FIG. 4. A barium swallow shoiuing the right colonic segment in the retrosternal position. VOL. 4, NO. I, JULY,

WALL, ESPINAS, SILVER, AND BYRON placed below the diaphragm. The postoperative course was stormy, requiring tracheostomy and mechanically assisted ventilation for a period of two weeks.

5 WALL, ESPINAS, SILVER, AND BYRON placed below the diaphragm. The postoperative course was stormy, requiring tracheostomy and mechanically assisted ventilation for a period of two weeks. Gradual improvement followed, and the patient was discharged on November 24, In December a brief readmission was necessary for closed drainage of a left pleural empyema space. In November, 1966, the patient returned, and gastrointestinal continuity was restored by interposing the right colon between the cervical esophagus and the stomach through a retrosternal tunnel. At the present time the patient is able to swallow a regular diet without difficulty (Fig. 4). When the abdomen was explored, the distal stomach and small and large intestine were entirely normal. Pathological studies of the resected specimen showed no evidence of neoplasm. The wall of the esophagus consisted of normally situated but massively hypertrophied smooth muscle measuring 3 cm. in thickness near the gastroesophageal junction and narrowing to 1.5 cm. near its proximal end. The massive muscular hypertrophy extended well down past the gastric cardia. DISCUSSION Achalasia is a term used to describe a functional disorder of the esophagus with abnormal motility and failure of the distal esophageal sphincter mechanism to relax for food to pass into the stomach. The major features of this disorder are functional distal obstruction with dilatation of the proximal esophagus. There may be some hypertrophy of the smooth muscle wall, but characteristically this is not massive. Features in the case presented which differ from those of achalasia are the massive smooth muscle hypertrophy of the esophageal wall measuring 3 cm. in thickness and extension of this hypertrophy well down into the gastric cardia. The other group of conditions reported and reviewed by Sloper [9], Spencer and Hudson [lo], Johnstone [6], and Blank and Michael [3], including idiopathic diffuse muscular hypertrophy of the lower esophagus and diffuse spasm of the esophagus, were characterized by smooth muscle hypertrophy of the esophagus and were not associated with significant dilatation. The hypertrophy was only slight when compared to that seen in the present case, and it was limited to the esophagus. A case of giant leiomyoma of the esophagus with obstruction and proximal dilatation of the esophagus has been reported [6]. The present case differs from such a leiomyoma in that it was not a neoplasm. The thick wall was due to muscular hypertrophy, not neoplasia, and the muscle layers were clearly discernible. Because of its unique features, this long, dilated, and massively hypertrophied segment of esophagus and proximal stomach is considered an entity distinct from those described above. Because of its extent, rather radical surgical treatment was necessary for its control. In reviews of the literature on esophageal pathology, no similar case has been encountered. 64 THE ANNALS OF THORACIC SURGERY

6 CASE REPORT: CONCLUSIONS In some respects achalasia, massive leiomyoma, and diffuse hypertrophy of the esophagus are suggested in this unusual case, but in other respects, the massiveness of the hypertrophy and its involvement of the stomach make this lesion unique. A total esophagectomy and delayed reconstruction was required to treat the lesion adequately. For these reasons this unusual lesion is presented as an entity, a case of giant esophagus characterized by idiopathic massive hypertrophy and dilatation of the esophagus and proximal stomach. REFERENCES Barrett, N. R., and Franklin, R. H. Concerning the unfavorable late results of certain operations performed in the treatment of cardiospasm. Brit. J. Surg. 37: 194, Belsay, R. Functional diseases of the esophagus. J. Thorac. Cardiov. Surg. 52: 164, Blank, E., and Michael, T. Muscular hypertrophy of the esophagus: Report of a case with involvement of the entire esophagus. Pediatrics 32:596, Bockus, H. L. Gastroenterology (2d ed.). Philadelphia: Saunders, Clagett, 0. T., Moersch, H. J., and Fisher, A. Esophagogastrostomy in the treatment of cardiospasm. Surg. Gynec. Obstet. 81:440, Johnstone, A. S. Diffuse spasm and diffuse muscle hypertrophy of lower esophagus. Brit. J. Radiol. 33:723, Kenny, L. J. Giant intramural leiomyoma of the esophagus: A case report..j. Thorac. Surg. 26:93, Merendino, K. A., and Dillard, D. H. The concept of sphincter substitution by an interposed jejunal segment for anatomic and physiologic abnormalities at the esophagogastric junction. Ann. Surg. 142:486, Sloper, 1. C. Idiopathic diffuse muscular hypertrophy of the lower esophagus. Thorax 9:136, Spencer, R., and Hudson, T. L. Idiopathic muscular hypertrophy of the gastrointestinal tract in a child. Surgery 50:678, Sweet, R. H. Surgical treatment of achalasia of the esophagus. New Eng. J. Med. 254:87, Taber, R. E., and Ehrenhaft, J. L. Esophageal motility in cardiospasm. A.M.A. Arch. Surg. 69:154, VOL. 4, NO. 1, JULY,

A Multidisciplinary Approach to Esophageal Dysphagia: Role of the SLP. Darlene Graner, M.A., CCC-SLP, BRS-S Sharon Burton, M.D.

A Multidisciplinary Approach to Esophageal Dysphagia: Role of the SLP Darlene Graner, M.A., CCC-SLP, BRS-S Sharon Burton, M.D. What is the role of the SLP? Historically SLPs the preferred providers for