Ann Thorac Surg 2003;75:1310-1311
© 2003 The Society of Thoracic Surgeons
Case report
Ulcerated mucocele of the esophagus in a child
Leopoldo Martínez, MDa*,
Juan A. Tovar, MD, PhDa
a Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain
Accepted for publication September 28, 2002.
* Address reprint requests to Dr Martínez, Departamento de Cirugía Pediátrica, Hospital Universitario La Paz, Paseo de la Castellana 261, 28046 Madrid, Spain
e-mail: jatovar{at}hulp.insalud.es
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Abstract
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A mucocele of an excluded esophagus is an unusual complication of esophageal surgery for atresia in childhood. We present here a 13-year-old boy with an infected mucocele and briefly discuss the symptoms, management, and pathologic findings of this rare lesion.
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Introduction
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Esophageal atresia (EA) is treated by primary or delayed esophageal anastomosis. Only a few cases require esophageal replacement [1], and only in very unusual conditions is the distal segment closed at both ends and abandoned in the mediastinum. This is a dangerous procedure [2–4], and the present report illustrates that these dangers do exist after treatment of EA.
A 13-year-old boy was referred to us after 2 months of thoracic pain, respiratory distress, cough, and fever. He had been diagnosed at birth with coloboma, heart defects, atresia of the choanae, retardation of growth and development, genital and urinary abnormalities, and ear abnormalities (CHARGE) in association with esophageal atresia and distal tracheoesophageal fistula, right renal agenesis, undescended testicle, right coanal atresia, and ear deformity. His fistula was ligated and a primary esophageal anastomosis was performed at the referring hospital, but an anastomotic breakdown prompted a new operation. During this operation, which a cervical esophagostomy, gastrostomy, and closure of the lower esophageal end from both the thorax and the abdomen were performed, leaving the thoracic esophagus excluded. At the age of 4 years, a retroesternal esophageal replacement with transverse colon failed because of vascular insufficiency. Four years later, the digestive tract continuity could be reestablished by a left colon graft located in the presternal position because of the previous retroesternal and mediastinum approaches.
On admission, physical examination showed malnourishment (weight 24.5 kg, < percentile 3 for age), thoracic deformity with retraction of the right sternoclavicular junction, protrusion of the distal sternum, and bilateral rales. The abdomen was soft and nontender, with a palpable spleen that seemed to be enlarged or displaced downward. A plain thoracic roentgenogram showed a retrocardiac condensation, and a computed tomographic scan showed a left subphrenic abscess displacing the spleen downward, a left basal pulmonary condensation, and a 4-cm mediastinal cyst located between the heart and aorta (Fig 1)
and extending from the carina to the hiatal region (Fig 2).
A huge amount of pus was percutaneously drained from the abscess under computed tomographic control, and the clinical picture immediately improved.
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Fig 1. Computed tomographic scan of the thorax showing a 4-cm mediastinal cyst located between the heart and the aorta, a left basal pulmonary condensation, and a moderate pleural effusion.
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Fig 2. Two consecutive images of thoracic coronal reconstruction from an axial computed tomographic scan showing a mediastinal cyst (arrow) extending from the carina to the hiatal region, where it is in close contact with a left subphrenic abscess (arrowhead). H = height; R = right.
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The patient was operated upon 10 days after drainage. The mediastinum was approached through a left lower thoracotomy. The abandoned, excluded esophagus appeared as a fusiform, pink, thick-walled, fluid-filled structure that started at the level of the carina and extended into the abdomen through the hiatus. The vessels that it received from the aorta were divided. The cyst was dissected from the pericardium, and 35 mL of sterile opaline fluid was aspirated. The cyst was then dissected free and excised from the carina to the upper pole of the spleen, to which it was firmly attached. An ulcer could be seen at this location. The postoperative course was uneventful, and the patient remains asymptomatic 24 months after the operation with a weight of 42 kg (p3 to p10). The pathology report indicated that the cyst consisted of ulcerated gastric wall, with a perforated ulcer in close contact with the spleen.
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Comment
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Isolated (type I) esophageal atresia occurs in at least 7% of cases, and an additional 15% of the type III cases are of the"long-gap" variety with wide separation of both ends. Primary or delayed anastomosis may be impossible in these cases and esophageal replacement is then indicated [1]. The development of a mucocele in an excluded esophageal segment is one of the rarest complications of this technique in pediatric patients [5].
Mucoceles develop because of the accumulation of secretions within the closed esophageal segment, and they usually remain small and asymptomatic [5–7]. However, sometimes they enlarge and become infected and even ulcerated, as in our patient. Resection is the treatment of choice in symptomatic cases, and drainage is recommended as a preliminary approach if there is any infected collection [3, 8]. In our case, ulceration and chronic infection of the cyst led to pain and severe malnutrition. Two years after mucocele resection, this boy gained more than 12 kg and now eats quite normally.
Much better known are esophageal mucoceles after esophageal exclusion for corrosive burns or neoplastic diseases in adult patients. These mucoceles usually develop in cases of total gastric resection for necrosis and perforation of the stomach, and can cause tracheal compression. If their diameter exceeds 50 mm, they should be resected or diverted, whereas smaller cases can be observed or treated by roentgenography-guided puncture [2, 7, 9].
The finding of gastric mucosa within the esophagocele is not surprising [5]. In our patient, it could correspond to the extension of a small portion of secretory epithelium that was present since birth or was secondary to metaplasia of the esophageal lining. The secretion of this type of mucosa likely caused the ulcer and the subphrenic abscess many years after birth.
Leaving behind a bipolarly excluded esophageal segment is a potentially dangerous surgical technique. We strongly recommend the complete excision of the lower esophageal segment during esophageal replacement whenever possible. This observation of a symptomatic esophagocele after a follow-up of more than 10 years illustrates well the risk of such an inappropriate procedure.
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References
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- Spitz L., Ruangtrakool R. Esophageal substitution. Semin Pediatr Surg 1998;7:130-133.[Medline]
- Mannell A., Epstein B. Exclusion of the oesophagus: is this a dangerous manoeuvre?. Br J Surg 1984;71:442-445.[Medline]
- Olsen C.O., Hopkins R.A., Postlethwait R.W. Management of an infected mucocele occurring in a bypassed excluded esophageal segment. Ann Thorac Surg 1985;40:73-75.[Abstract/Free Full Text]
- Erasmus J.J., McAdams H.P., Goodman P.C. Diagnosis please. Case 5: esophageal mucocele after surgical bypass of the esophagus. Radiology 1998;209:757-760.[Free Full Text]
- Kamath M.V., Ellison R.G., Rubin J.W., Moore H.V., Pai G.P. Esophageal mucocele: a complication of blind loop esophagus. Ann Thorac Surg 1987;43:263-269.[Abstract/Free Full Text]
- Glickstein M.F., Gefter W.B., Low D., Stephenson L.W. Esophageal mucocele after surgical isolation of the esophagus. Am J Roentgenol 1987;149:729-730.[Free Full Text]
- Chambon J.P., Robert Y., Remy J., Ribet M. Mucoceles esophagiennes compliquant la double exclusion de l’esophage après ingestion de caustique. Ann Chir 1989;43:724-730.[Medline]
- Saunders A.M., Swayne L.C., Schwartz J.R., Moritz M.W. Mediastinal mass following esophageal surgery. N J Med 1991;88:571-572.[Medline]
- Ribet M., Chambon J.P., Pruvot F.R. Oesophagectomy for severe corrosive injuries: is it always legitimate?. Eur J Cardio-thorac Surg 1990;4:347-349.[Abstract/Free Full Text]
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Abstract
Introduction
