Ann Thorac Surg 1997;63:531-533
© 1997 The Society of Thoracic Surgeons
Case Report
Esophageal Leiomyomatosis Involving Trachea: Surgical Resection and Repair
Pankaj Kumar, BM, BCh,
Nicholas M. Breach, FRCS,
Peter Goldstraw, FrcS.
Department of Surgery, Royal Brompton Hospital, London, United Kingdom
Accepted for publication August 21, 1996.
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Abstract
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We report the case of a 26-year-old man with diffuse esophageal leiomyomatosis involving the trachea. The tumor was resected by total esophagectomy and partial resection of the trachea and the left main bronchus. The tracheobronchial defect was repaired with a free forearm skin graft with satisfactory outcome. This approach offers good long-term prospects.
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Introduction
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A 26-year-old man presented with a long history of dysphagia. He was unable to swallow solids and experienced regurgitation. Ten years previously, he had an operation abroad, after which he was asymptomatic for 2 years. Unfortunately no operative or pathologic details were available. Physical examination was unremarkable. Biochemical and hematologic profiles were normal. Spirometry suggested obstructive lung disease (forced expiratory volume in 1 second/forced vital capacity of 1.5/2.8). A widened and distorted mediastinal contour was noted on the chest roentgenogram (Fig 1
). Barium swallow showed an irregular esophageal contour from the level of T-3 to the gastroesophageal junction, with several discrete areas of narrowing and filling defects (Fig 2). Radiopaque solids demonstrated hold-up at the most proximal narrowing, and esophageal peristalsis was abnormal. A computed tomographic scan showed the esophageal wall below T-3 to be thickened and reducing the esophageal lumen to a pinpoint at the level of the carina. Further, there was significant narrowing of the left main bronchus. At bronchoscopy, the lower trachea and left main bronchus were seen to be compressed with abnormal and bosselated mucosa, suggesting involvement by tumor from behind. Esophagoscopy revealed grossly abnormal esophagus with submucosal tumor protruding into the esophageal lumen.
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Fig 1. . Chest roentgenogram showing the widened mediastinal contour (arrow) and the oligemic left lung.
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Fig 2. . Barium swallow demonstrating the widened esophagus and irregular esophageal wall with multiple filling defects.
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Histologic examination of the tracheal biopsy specimen showed nodules of cytologically bland spindle cells with esoinophilic cytoplasm. The esophageal biopsy specimen showed nonkeratinizing squamous epithelium covering mucosal nodules similar to those seen in the tracheal biopsy specimen (Fig 3). The appearances suggested multifocal leiomyoma of esophagus involving the posterior wall of the trachea.
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Fig 3. . Microscopic appearance of an esophageal nodule showing sheets of cytologically benign spindle cells (CS). Nonsquamous epithelium (NKSE) covering the nodules is also seen. (Hematoxylin and eosin stain.)
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Two-stage Ivor Lewis esophagectomy was performed. Multilobulated tumor was seen in the esophageal wall from the apex of the pleural space to the diaphragm (Fig 4). The esophagus was dissected free from the trachea, but a ridge of abnormal tissue remained on the posterior wall of the trachea and left main bronchus, which was excised as a flap from the membranous part of the tracheobronchial tree. An attempt was made to ventilate the left lung across the operating field. The left lung could not support satisfactory gas exchange. At this stage the chest roentgenogram was reviewed and the presence of McCloud's syndrome (oligemic lung) was recognized (see Fig 1
). Therefore the rest of the operation was performed with intermittent ventilation with operation during periods of apnea until hypoxia necessitated interruption of the operation for ventilation of the right lung. A free forearm skin flap (5 cm x 2 cm) based on the radial artery and cephalic vein was raised, transferred to the thoracic cavity, and anastomosed to the right internal mammary artery (interrupted 8/0 Prolene [Ethicon, Edinburgh, Scotland]) and superior vena cava (continuous 8/0 Prolene), both in an end-to-side manner. The free skin flap was sewn over the posterior tracheobronchial defect using 4/0 Prolene. Esophagectomy was then completed and the esophagogastric anastomosis was performed using continuous 3/0 Prolene. The omentum was wrapped around and sewn over the tracheal repair, the gastrotomy closure, and the anastomosis. Postoperative recovery was slow due to contusion of the right lung during the operation but otherwise uneventful. One month after the operation the patient had no dysphagia.
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Fig 4. . Macroscopic appearance of the resected esophagus showing multiple nodules in the esophageal wall.
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Macroscopically, the esophagus and trachea were thickened by several foci of confluent cream-colored nodules measuring up to 35 mm in diameter (see Fig 4). Microscopically, these nodules consisted of sheets of cytologically benign spindle cells (see Fig 3). The spindle cells stained positive for desmin, a smooth muscle marker. No mitotic activity or nuclear atypia was seen, thereby suggesting a low level of malignant potential. Lymph nodes and resection margins were free of tumor.
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Comment
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Although tumors of the esophagus involving the trachea would normally be considered inoperable, in view of the very low grade of malignancy and the young age of the patient, the only prospect for satisfactory long-term result would be esophagectomy with tracheal resection and reconstruction. The alternative approaches would have provided no prospect for cure and resulted in a shorter survival with compromised quality of life. This case highlights several important points: multifocal leiomyomatosis of the esophagus and its potential to involve adjacent structures, tracheobronchial resection and repair of the defect with a free skin graft, and the unexpected finding of McCloud's syndrome.
Esophageal leiomyomas are rare, benign tumors of smooth muscle origin and represent only 0.4% of all esophageal tumors. Multiple esophageal leiomyomatosis is even rarer [1]. Only a few cases of multiple leiomyomatosis have been reported. Multiple esophageal leiomyomatosis is associated with Alport's syndrome (deafness, cataract, and hematuria) [2]. Familial leiomyomatosis of the esophagus in combination with or without nephropathy affecting children has been described and is believed to be inherited dominantly [3]. The case presented had none of the features of Alport's syndrome, and all other family members were asymptomatic, although they were not investigated.
Leiomyomas have a low potential for malignant transformation, and whether or not the esophageal leiomyomas can metastasize is not known. However, there is much better understanding of the natural history of uterine leiomyoma, and these are capable of vascular invasion [4], intracardiac extension [4], disseminated intraperitoneal involvement [5], and metastasizing to lungs [6]. Based on this experience, multifocal esophageal leiomyomatosis should definitely be treated as having a low probability of being malignant.
In this case, surgical intervention was necessary for symptomatic reasons. However, involvement of the posterior aspect of the major airway presented a problem. Multiple esophageal leiomyomatosis causing severe dysphagia necessitated total esophagectomy, although this alone would have been inadequate resection. In this case, the posterior wall of the trachea and left main bronchus had been involved by the tumor, and a surgical approach offered the only chance of a cure. The abnormal tissue from the tracheobronchial tree could be resected and the defect repaired with a free skin graft. The radial forearm flap was used as it is a donor site where the skin is thin and pliable and has reliable vascularity. In our case the chosen skin island was hairless, although hairs do occasionally present a problem. Alternatives for tracheal repair would be to stitch the wall of the esophageal replacement to the tracheal defect or to undertake bronchoplastic tracheobronchial reconstruction. In this case an "independent" repair was favored.
The unexpected finding of oligemic left lung representing McCloud's syndrome only made the ventilation and operation more challenging. Whether the oligemic left lung was a consequence of the earlier left thoracotomy or a congenital feature remains unclear. If this finding had been noted preoperatively, cardiopulmonary bypass would have made the operation less arduous and prevented the contusion of the right lung.
This case serves as a reminder that even so-called benign tumors such as leiomyomas are capable of involving adjacent structures. It also describes successful repair of a tracheobronchial defect by free skin grafts. As shown in this case, this approach has a satisfactory early result and offers another alternative in tracheobronchial reconstruction. We await review of the long-term outcome.
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Footnotes
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Address reprint requests to Mr Goldstraw, Department of Surgery, Royal Brompton Hospital, Sydney St, London, SW3 6NP, United Kingdom.
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References
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- Seremetis MG, Lyons WS, de Guzman VC, Peabody JW Jr. Leiomyomata of the esophagus. An analysis of 838 cases. Cancer 1976;38:2166–77.[Medline]
- Rabushka LS, Fishman EK, Kuhlman JE, Hruban RH. Diffuse esophageal leiomyomatosis in a patient with Alport syndrome: CT demonstration. Radiology 1991;179:176–8.[Abstract/Free Full Text]
- Lonsdale RN, Roberts PF, Vaughan R, Thiru S. Familial oesophageal leiomyomatosis and nephropathy. Histopathology 1992;20:127–33.[Medline]
- To WW, Ngan HY, Collins RJ. Intravenous leiomyomatosis with intracardiac involvement. Int J Gynaecol Obstet 1993;42:37–40.[Medline]
- Gana BM, Byrne J, McCullough J, Weaver JP. Leiomatosis peritonealis disseminata with associated endometriosis: a case report. J R Coll Surg Edinb 1994;39:258–60.
- Vallina E, Rodriguez VM, Gonzalez LD, et al. A case of uterine leiomyoma with pulmonary metastasis. An Med Int 1994;11:129–32.
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Abstract
Introduction
