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Bile ptyalism in a patient with sickle cell anemia simulating bronchobiliary fistula
Bile Ptyalism in a Patient with Sickle Cell Anemia Simulating Bronchobiliary Conservative
Fistula
Management
James A. Christensen, MD, Tampa, Florida Roger T. Sherman, MD, Tampa, Florida Richard H. Blank, MD, Tampa, Florida
Bile ptyalism usually indicates the presence of a bronchobiliary fistula. Various causes of bile in the sputum in the adult include trauma, hepatic disease, and biliary obstruction [l-7]. In the newborn, bile ptyalism may be due to congenital connections between the pulmonary and biliary systems [8,9]. In rare instances bile ptyalism can also be due to sickle cell crisis or sickle cell disease [IO]. Since most causes of bile ptyalism require surgical management, it is important to be aware of occasional nonsurgical causes of this condition. The opportunity to treat a patient with sickle cell anemia and associated bile ptyalism has prompted the following report. Case Report The patient was admitted complaints of duration. The
(EP), a twenty-two year old black to the Tampa General Hospital with chest pain, chills, and fever of four chest pain was on the right side, was
man, chief days’ pleu-
From the Department of Surgery, University of South Florida College of Medicine, Tampa General Hospital, Tampa, Florida. Reprint requests should be addressed to Dr Sherman, Department of Surgery, University of South Florida College of Medicine, Tampa General Hospital, Tampa, Florida 33606.
Volume
127.
June
1974
ritic in nature, and was associated with pain in the right upper abdominal quadrant. The cough was productive of thin, golden yellow sputum. The patient had a long history of sickle cell anemia with several episodes of crisis. He had recently been discharged from the Marines because of the sickle cell disease. During active duty he spent time in Okinawa. There was a history of blunt trauma to the right upper quadrant of the abdomen when a tar cart ran over him six months prior to this admission. He was hospitalized for observation after this accident and was noted to have fractures of the ninth and tenth ribs on the right. His family history was significant in that his father and brother died of sickle cell disease. On examination, temperature was 102.2’F, pulse 120, and respirations 29 per minute. The conjunctivae were icteric. Examination of the chest demonstrated both dullness to percussion and decreased breath sounds over the right lower part of the chest. The abdomen was tender in the right upper quadrant and the liver was palpable 3 cm below the right costal margin. Chest x-ray film disclosed a right pleural effusion with patchy infiltrates of the lower lobe of the right lung. The findings on examination of the chest, chest x-ray film (Figure l), and golden yellow sputum led t.o a preliminary diagnosis of bronchobiliary fistula. Examination of the sputum showed gram-negative rods with many white blood cells. Sputum bilirubin was 7 mg per
765
Christensen, Sherman, and Blank
Primary
tuberculous
liver abscess, a rare findas a cause of bronchobiliary fistula [3]. The mechanism involved was a subphrenic collection that ruptured through the diaphragm into a bronchus. Drainage and closure of the diaphragm and the fistula are the indicated treatment. Hodgkin’s disease of the liver with granuloma formation and secondary necrosis causing a subphrenic collection of debris has been reported as a cause of bronchobiliary fistula [7]. Pyogenic abscesses of the liver can erode through the diaphragm and communicate with the bronchial tree [2]. Drainage combined with antibiotic therapy will adequately control this problem. Inadequate drainage of liver wounds secondary to blunt or penetrating trauma can lead to subphrenic collections of bile. These collections associated with infection or diaphragmatic injury can lead to communication with the bronchi [2]. Iatrogenic trauma from liver biopsy and therapeutic induction of pneumoperitoneum have been reported as causes of bronchobiliary fistula [2,1 I]. Obstruction of the biliary system by stones, neoplasm, gumma, tuberculosis, stricture, and ascaris lumbricoides has been reported as a cause of bronchobiliary fistula [1,5]. Most of these cases were associated with suppurative processes. Congenital bronchobiliary fistula is a rare cause of respiratory distress in the newborn [8,9]. Infants with congenital fistula have cough, productive of yellow or green sputum, beginning in the first few days of life. There are two possible explanations for congenital fistula formation. Union of an anomalous bronchial bud with an anomalous bile duct is one possibility. The other is duplication of the upper gastrointestinal tract in that a foregut duplication from the laryngotracheal outgrowth unites with a hepatic diverticulum forming a bronchobiliary fistula. Operative management of bronchobiliary fistula should include thoracotomy, adequate subcostal drainage of the hepatic bed under direct vision, secure closure of the diaphragmatic perforation, and, if necessary, lobectomy. Decompression of the biliary tree with a T tube should be considered [2]. The triad of sickle cell anemia, chest trauma, and bile ptyalism has previously been reported without evidence of fistula [IO]. Sickle cell hemoglobinopathy can cause bile-containing sputum. Pulmonary infection and infarction are common complications of sickle cell disease. It is possible that bile ptyalism is a complication of these changes in the lung. However, the exact cause of
ing, has been reported
Figure 1. Chest x-ray film showing infiltrate of lower lobe of right lung.
100 ml. Two hundred cubic centimeters of yellow fluid was obtained by thoracentesis of the right pleural cavity. Pleural fluid biliruhin was 4.5 per cent. Serum bilirubin was 7.0 mg per 100 ml with a direct component of 1.8 mg per 100 ml. Hemoglobin on admission was 9.6 gm per 100 ml and the hematocrit was 29 per cent. He was treated with antibiotics and corticosteroids. Bronchoscopy revealed erythema of the right main stem bronchus. Copious amounts of yellow sputum were aspirated from this location. Bronchograms taken two days later showed no communication with the biliary tract. Four days after admission the productive cough subsided. Liver-lung scan showed nothing abnormal; rose bengal study of the liver and sputum gave negative results. He became afebrile and the pneumonia gradually cleared as determined by x-ray film. He signed out against medical advice eleven days after admission. Comments
Although uncommon in the United States, echinococcus disease of the liver probably is the leading cause of bronchobiliary fistula. It is caused by a tapeworm parasite found in dogs, wolves, and sometimes cats [4]. Treatment of the cyst is evacuation and cauterization of the cyst wall or irrigation with formalin. Amebiasis is encountered in Southern border states and pulmonary complications including bronchobiliary fistula have been reported [2,6].
766
The American Journal of Surgery
Bile Ptyalism
copious bilious sputum in sickle cell disease is unknown. Adequat.e evaluation including bronchoscopy, hronchography, liver-lung scan, and rose bengal study of liver and sputum should be carried out. If no fistula can be demonstrated, medical management of the crisis and pneumonitis should be continued. In our case, the progressive pneumonia was treated with steroids to decrease the irritating effect. of bile. Also added to this patient’s conservative treatment were broad spectrum antibiotics. Summary
Bronchobiliary fistula is the primary diagnosis to be considered in a patient with bile ptyalism. A case of bile ptyalism associated with sickle cell disease and crisis is presented, providing a rare instance in which nonoperative management is indicated in conjunction with this symptom.
Volume 127, June 1974
and Sickle
Cell Anemia
References 1. Adams
HD: Pleurobiliary
and bronchobiliary
fistulas.
J Thorac
Cardiovasc Surg30: 255, 1955. 2. Ferguson TB, Burford TH: Pleurobiliary and bronchobiliary fistulas. Arch Surg95: 380, 1967. 3. Flemma RJ, Anlyan WG: Tuberculous bronchobiliary fistula.
J
Thorac Cardiovasc Surg 49: 198, 1965 4. Judd ES: Surgical treatment for echinococcus cysts of the liver: report of four cases. Mayo C/in Proc 35: 58, 1960. 5. Morton JJ. Phillips EW: Bronchobiliary fistula. Arch Surg 16: 694, 1928. 6. Roy DC, Ravindran P. Padmanabhan R: Bronchobiliary fistula secondary to amebic liver abscess. Chest62: 523, 1972. 7. Trubowitz S: Bronchobiliary fistula in Hodgkin’s disease. Arch intern Med 88: 400, 195 1. 8. Sane SM, Sieber WK, Girdany BR: Congenital bronchobiliary fistula. Surgery 69: 599, 197 1. 9. Wagget J. Stool S, Bishop HC, Kurtz MB: Congenital bronchobiliary fistula. J fediatr Surg 5: 566, 1970. 10. Sutherland RD, Reynolds J. Sugg WL: Bile ptyalism associated with chest trauma and sickle cell crisis simulating 11.
bronchobiliary fistula. Ann Thorac Surg 13: 537, 1972. Mansour KA, Black WS. Dominy DE, Abbott OA: A new cause for biliobronchial fistula. Dis Chest 56: 539,
1969.
767
Fistula
Management
James A. Christensen, MD, Tampa, Florida Roger T. Sherman, MD, Tampa, Florida Richard H. Blank, MD, Tampa, Florida
Bile ptyalism usually indicates the presence of a bronchobiliary fistula. Various causes of bile in the sputum in the adult include trauma, hepatic disease, and biliary obstruction [l-7]. In the newborn, bile ptyalism may be due to congenital connections between the pulmonary and biliary systems [8,9]. In rare instances bile ptyalism can also be due to sickle cell crisis or sickle cell disease [IO]. Since most causes of bile ptyalism require surgical management, it is important to be aware of occasional nonsurgical causes of this condition. The opportunity to treat a patient with sickle cell anemia and associated bile ptyalism has prompted the following report. Case Report The patient was admitted complaints of duration. The
(EP), a twenty-two year old black to the Tampa General Hospital with chest pain, chills, and fever of four chest pain was on the right side, was
man, chief days’ pleu-
From the Department of Surgery, University of South Florida College of Medicine, Tampa General Hospital, Tampa, Florida. Reprint requests should be addressed to Dr Sherman, Department of Surgery, University of South Florida College of Medicine, Tampa General Hospital, Tampa, Florida 33606.
Volume
127.
June
1974
ritic in nature, and was associated with pain in the right upper abdominal quadrant. The cough was productive of thin, golden yellow sputum. The patient had a long history of sickle cell anemia with several episodes of crisis. He had recently been discharged from the Marines because of the sickle cell disease. During active duty he spent time in Okinawa. There was a history of blunt trauma to the right upper quadrant of the abdomen when a tar cart ran over him six months prior to this admission. He was hospitalized for observation after this accident and was noted to have fractures of the ninth and tenth ribs on the right. His family history was significant in that his father and brother died of sickle cell disease. On examination, temperature was 102.2’F, pulse 120, and respirations 29 per minute. The conjunctivae were icteric. Examination of the chest demonstrated both dullness to percussion and decreased breath sounds over the right lower part of the chest. The abdomen was tender in the right upper quadrant and the liver was palpable 3 cm below the right costal margin. Chest x-ray film disclosed a right pleural effusion with patchy infiltrates of the lower lobe of the right lung. The findings on examination of the chest, chest x-ray film (Figure l), and golden yellow sputum led t.o a preliminary diagnosis of bronchobiliary fistula. Examination of the sputum showed gram-negative rods with many white blood cells. Sputum bilirubin was 7 mg per
765
Christensen, Sherman, and Blank
Primary
tuberculous
liver abscess, a rare findas a cause of bronchobiliary fistula [3]. The mechanism involved was a subphrenic collection that ruptured through the diaphragm into a bronchus. Drainage and closure of the diaphragm and the fistula are the indicated treatment. Hodgkin’s disease of the liver with granuloma formation and secondary necrosis causing a subphrenic collection of debris has been reported as a cause of bronchobiliary fistula [7]. Pyogenic abscesses of the liver can erode through the diaphragm and communicate with the bronchial tree [2]. Drainage combined with antibiotic therapy will adequately control this problem. Inadequate drainage of liver wounds secondary to blunt or penetrating trauma can lead to subphrenic collections of bile. These collections associated with infection or diaphragmatic injury can lead to communication with the bronchi [2]. Iatrogenic trauma from liver biopsy and therapeutic induction of pneumoperitoneum have been reported as causes of bronchobiliary fistula [2,1 I]. Obstruction of the biliary system by stones, neoplasm, gumma, tuberculosis, stricture, and ascaris lumbricoides has been reported as a cause of bronchobiliary fistula [1,5]. Most of these cases were associated with suppurative processes. Congenital bronchobiliary fistula is a rare cause of respiratory distress in the newborn [8,9]. Infants with congenital fistula have cough, productive of yellow or green sputum, beginning in the first few days of life. There are two possible explanations for congenital fistula formation. Union of an anomalous bronchial bud with an anomalous bile duct is one possibility. The other is duplication of the upper gastrointestinal tract in that a foregut duplication from the laryngotracheal outgrowth unites with a hepatic diverticulum forming a bronchobiliary fistula. Operative management of bronchobiliary fistula should include thoracotomy, adequate subcostal drainage of the hepatic bed under direct vision, secure closure of the diaphragmatic perforation, and, if necessary, lobectomy. Decompression of the biliary tree with a T tube should be considered [2]. The triad of sickle cell anemia, chest trauma, and bile ptyalism has previously been reported without evidence of fistula [IO]. Sickle cell hemoglobinopathy can cause bile-containing sputum. Pulmonary infection and infarction are common complications of sickle cell disease. It is possible that bile ptyalism is a complication of these changes in the lung. However, the exact cause of
ing, has been reported
Figure 1. Chest x-ray film showing infiltrate of lower lobe of right lung.
100 ml. Two hundred cubic centimeters of yellow fluid was obtained by thoracentesis of the right pleural cavity. Pleural fluid biliruhin was 4.5 per cent. Serum bilirubin was 7.0 mg per 100 ml with a direct component of 1.8 mg per 100 ml. Hemoglobin on admission was 9.6 gm per 100 ml and the hematocrit was 29 per cent. He was treated with antibiotics and corticosteroids. Bronchoscopy revealed erythema of the right main stem bronchus. Copious amounts of yellow sputum were aspirated from this location. Bronchograms taken two days later showed no communication with the biliary tract. Four days after admission the productive cough subsided. Liver-lung scan showed nothing abnormal; rose bengal study of the liver and sputum gave negative results. He became afebrile and the pneumonia gradually cleared as determined by x-ray film. He signed out against medical advice eleven days after admission. Comments
Although uncommon in the United States, echinococcus disease of the liver probably is the leading cause of bronchobiliary fistula. It is caused by a tapeworm parasite found in dogs, wolves, and sometimes cats [4]. Treatment of the cyst is evacuation and cauterization of the cyst wall or irrigation with formalin. Amebiasis is encountered in Southern border states and pulmonary complications including bronchobiliary fistula have been reported [2,6].
766
The American Journal of Surgery
Bile Ptyalism
copious bilious sputum in sickle cell disease is unknown. Adequat.e evaluation including bronchoscopy, hronchography, liver-lung scan, and rose bengal study of liver and sputum should be carried out. If no fistula can be demonstrated, medical management of the crisis and pneumonitis should be continued. In our case, the progressive pneumonia was treated with steroids to decrease the irritating effect. of bile. Also added to this patient’s conservative treatment were broad spectrum antibiotics. Summary
Bronchobiliary fistula is the primary diagnosis to be considered in a patient with bile ptyalism. A case of bile ptyalism associated with sickle cell disease and crisis is presented, providing a rare instance in which nonoperative management is indicated in conjunction with this symptom.
Volume 127, June 1974
and Sickle
Cell Anemia
References 1. Adams
HD: Pleurobiliary
and bronchobiliary
fistulas.
J Thorac
Cardiovasc Surg30: 255, 1955. 2. Ferguson TB, Burford TH: Pleurobiliary and bronchobiliary fistulas. Arch Surg95: 380, 1967. 3. Flemma RJ, Anlyan WG: Tuberculous bronchobiliary fistula.
J
Thorac Cardiovasc Surg 49: 198, 1965 4. Judd ES: Surgical treatment for echinococcus cysts of the liver: report of four cases. Mayo C/in Proc 35: 58, 1960. 5. Morton JJ. Phillips EW: Bronchobiliary fistula. Arch Surg 16: 694, 1928. 6. Roy DC, Ravindran P. Padmanabhan R: Bronchobiliary fistula secondary to amebic liver abscess. Chest62: 523, 1972. 7. Trubowitz S: Bronchobiliary fistula in Hodgkin’s disease. Arch intern Med 88: 400, 195 1. 8. Sane SM, Sieber WK, Girdany BR: Congenital bronchobiliary fistula. Surgery 69: 599, 197 1. 9. Wagget J. Stool S, Bishop HC, Kurtz MB: Congenital bronchobiliary fistula. J fediatr Surg 5: 566, 1970. 10. Sutherland RD, Reynolds J. Sugg WL: Bile ptyalism associated with chest trauma and sickle cell crisis simulating 11.
bronchobiliary fistula. Ann Thorac Surg 13: 537, 1972. Mansour KA, Black WS. Dominy DE, Abbott OA: A new cause for biliobronchial fistula. Dis Chest 56: 539,
1969.
767