Ann Thorac Surg 1999;68:2338-2339
© 1999 The Society of Thoracic Surgeons
Case Reports
Communicating bronchopulmonary pancreatic foregut malformation
G. Farah Rahman, MDb,
Nikhil Bhardwaj, MDc,
Bernard Suster, MDd,
Jeffrey J. Arliss, MDb,
Cliff P. Connery, MDa
a Division of Cardiothoracic Surgery, St. Luke’s–Roosevelt Hospital Center, Columbia University College of Physicians and Surgeons, New York, New York, USA
b Department of Surgery, St. Luke’s–Roosevelt Hospital Center, Columbia University College of Physicians and Surgeons, New York, New York, USA
c Department of Pulmonary Medicine, St. Luke’s–Roosevelt Hospital Center, Columbia University College of Physicians and Surgeons, New York, New York, USA
d Department of Radiology, St. Luke’s–Roosevelt Hospital Center, Columbia University College of Physicians and Surgeons, New York, New York, USA
Address reprint requests to Dr Connery, Division of Cardiothoracic Surgery, St. Luke’s–Roosevelt Hospital Center, 1111 Amsterdam Ave, New York, NY 10025
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Abstract
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Bronchopulmonary foregut malformations include intralobar and extralobar pulmonary sequestrations, bronchogenic cysts, and communicating bronchopulmonary foregut malformations (CBPFM). These malformations, formes frustes, originate as developmental abnormalities of ventral foregut budding of the tracheobronchial tree or the gastrointestinal tract. The communication’s patency with the parent viscus determines if a contained malformation occurs, or if an abnormal communication persists as a CBPFM. This case demonstrates a unique example of a CBPFM in which the main pancreatic duct communicated with pulmonary parenchyma through a retroperitoneal fistula.
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Introduction
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This case demonstrates a unique example of a communicating bronchopulmonary foregut malformation, formes frustes, in which the main pancreatic duct communicated with pulmonary parenchyma via a retroperitoneal fistula.
The patient is a 36-year-old woman who had previously undergone right thoracotomy for a cyst resection 23 years ago. Two years after this procedure, and recently, the patient presented on multiple occasions for shortness of breath, scant hemoptysis, and cough. Sputum cultures yielded strep pyogenes. Review of systems is notable for a history of smoking (tobacco) and cocaine snorting. The patient was tested human immunodeficiency virus and purified protein derivative (PPD) negative. The patient further reported 40 lbs weight loss over 1 year. Prior to the current admission, she denied any fevers or night sweats. Vitals on presentation were: temperature of 37°C, a respiratory rate of 20, and a room air oxygen saturation of 96%. A right anterolateral thoracotomy scar was evident on exam as were decreased right breath sounds and clubbing. The white blood cell count was 7.6 with a normal differential. The chest radiograph (CXR) demonstrated a right paramediastinal cyst, and right-sided infiltrates. Bronchoscopy revealed hyperemic airways. Cytology was negative. Computerized tomogram (CT) was performed. It further demonstrated a right lower lobe cyst containing multiple air fluid levels, with adjacent lung showing numerous cystic lesions, air space disease, and fibrosis. Mediastinal adenopathy was present as well.
Consultations from gastroenterology and general surgery were obtained prior to thoracotomy. However, enteric communication was not identified by contrast CT scan, upper gastrointestinal series, or magnetic resonance imaging.
The patient subsequently underwent right thoracotomy, an extensive lysis of adhesions, right middle lobectomy and lower lobectomy for extensively destroyed pulmonary parenchyma (from repeated pneumonias), mediastinal node dissection, and cyst wall biopsy. No collections or masses were present. Intraoperative pathology of the mediastinal lymph nodes demonstrated benign reactive lymphoid follicular hyperplasia. The lung specimen demonstrated chronic inflammatory changes, destruction, and extensive scarring. Cell count, chemistries, and cytology of the cyst contents were not performed. Biopsy of the cyst wall demonstrated fibrous changes and focal mucous glands. Further, this epithelialized cyst was noted to extend across the diaphragm for approximately 5 cm along the inferior vena cava. Given the absence of malignancy on frozen section, and the presence of vascular adherence and infradiaphragmatic extension, no further resection was carried out at initial surgery.
On postoperative day 13, the patient developed fevers, leukocytosis, purulent sputum, and a right pleural effusion. After a diagnostic thoracentesis, a chest tube was placed for this new right pleural effusion. The fluid cell count was 100% neutrophils. The pH was 7.0, the amylase was 121,440, and the lipase was 5,200. Concurrent serum amylase and lipase were 177 and 63, respectively. An endoscopic retrograde cholangiopancreatography (ERCP) demonstrated a fistula connecting the main pancreatic duct with the right pleural space.
The patient subsequently underwent laparotomy and distal pancreatectomy. The distal pancreas was transected with a scalpel. The fistula and pancreatic duct were identified. Their common channel at the distal pancreas was cannulated with an 18-gauge angiocath, and an intraoperative pancreaticogram was performed (Fig 1).
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Fig 1. Intraoperative pancreaticogram shows a pancreatic fistula extending superiorly towards the mediastinum and right lower lung.
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Postoperatively, the patient defervesced. She was discharged with uneventful 6-month follow-up. Pathology showed normal ductal epithelium lining the fistulous tract. The pancreatic parenchyma in the resected specimen was normal.
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Comment
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Although congenital bronchopulmonary foregut malformations are generally identified in the pediatric population, delayed adolescent presentations have been reported [1]. Pneumonias associated with cystic radiographic structures, especially in the lower lobes, are suggestive of the diagnosis. Further imaging using esophogram, bronchography, computerized tomography, and arteriography aid in the diagnostic evaluation [2]. Pleural investment and systemic arterial supply are not constant. The treatment is surgical resection of the involved lung parenchyma, and fistula closure [3].
This report demonstrates a unique CBPFM. Most enteric communications are with the distal esophagus or stomach. Consistent with the formes frustes hypothesis, this unique pancreatic communication represents the distal, preserved foregut origin of the malformation [4]. Focal mucous glands as in this cyst, or cells of gastric origin lining the cyst have been described [5]. Heterotopic pancreatic tissue has also been described [4].
In this patient, we believe the pancreatic exocrine secretions contributed to the distal lung parenchymal destruction, and multiple pneumonias. Fistula management using pancreatectomy, and resection of the extensively destroyed lung were critical steps in limiting pulmonary contamination.
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References
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Schwartz M.Z., Ramachandran P. Congenital malformations of the lung and mediastinum. J Pediatr Surg 1997;32:44-47.[Medline]
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Kravitz R.M. Congenital malformations of the lung. Pediatr Clin North Am 1994;41:453-472.[Medline]
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Nuchtern J.G., Harberg F.J. Congenital lung cysts. Semin Pediatr Surg 1994;3:233-243.[Medline]
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Tilson M.D., Touloukian R.J. Mediastinal enteric sequestration with aberrant pancreas. Ann Surg 1972;176:669-671.[Medline]
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Ferguson T.B., Jr, Ferguson T.B. Congenital lesions of the lung and emphysema. In: Sabiston D.C., Jr, Spencer F., eds. Surgery of the chest. Philadelphia: Saunders, 1995:822-884.
Accepted for publication May 9, 1999.
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Abstract
Introduction
