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Surgical Experience in a Baby With Congenital Broncho-Biliary Fistula

2008; Elsevier BV; Volume: 87; Issue: 1 Linguagem: Inglês

10.1016/j.athoracsur.2008.06.028

1552-6259

Ayla Günlemez, Melih Tugay, Levent Elemen, Gülcan Türker, Nagihan İnan Gürcan, Hakan Demir, Yeşim Gürbüz, Tülay Hoşten,

Pediatric Hepatobiliary Diseases and Treatments

Congenital tracheobiliary and bronchobiliary fistulae are rare malformations in which patent communications exist between the respiratory system and biliary tract, respectively. We present a newborn who was admitted with respiratory distress and bilious tracheal discharge. Investigation revealed a bronchobiliary fistula originating from the left main bronchus, as well as biliary atresia. Excision of the bronchobiliary fistula was successful and the connection between biliary tract and gastrointestinal system was established by performance of a Roux-en-Y cholecysto-jejunostomy. Diagnostic tools, differential diagnosis, and surgical correction strategies are discussed. Congenital tracheobiliary and bronchobiliary fistulae are rare malformations in which patent communications exist between the respiratory system and biliary tract, respectively. We present a newborn who was admitted with respiratory distress and bilious tracheal discharge. Investigation revealed a bronchobiliary fistula originating from the left main bronchus, as well as biliary atresia. Excision of the bronchobiliary fistula was successful and the connection between biliary tract and gastrointestinal system was established by performance of a Roux-en-Y cholecysto-jejunostomy. Diagnostic tools, differential diagnosis, and surgical correction strategies are discussed. Congenital respiratory system biliary tract fistula is a rare developmental anomaly. It has been reported in conjunction with other congenital anomalies, such as biliary tract malformations, diaphragmatic hernia, and esophageal atresia [1Tekant G.A. Joseph V.T. Cheah S.L. Congenital tracheobiliary fistula.J Pediatr Surg. 1994; 29: 594-595Abstract Full Text PDF PubMed Scopus (17) Google Scholar, 2Egrari S. Krishnamoorthy M. Yee C.A. Applebaum H. Congenital bronchobiliary fistula: diagnosis and postoperative surveillance with HIDA scan.J Pediatr Surg. 1996; 31: 785-786Abstract Full Text PDF PubMed Scopus (27) Google Scholar, 3Kalayoglu M. Olcay I. Congenital bronchobiliary fistula associated with esophageal atresia and tracheo-esophageal fistula.J Pediatr Surg. 1976; 11: 463-464Abstract Full Text PDF PubMed Scopus (33) Google Scholar]. Various types of tracheobiliary or bronchobiliary fistulae (BBF) have been described [4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar]. A 9-day-old female infant was admitted with respiratory distress and bilious vomiting. Her medical history disclosed that the patient had experienced bilious vomiting and subsequent respiratory distress on the postnatal third hour. Physical examination revealed yellow colored salivation and diminished lung sounds on the left hemithorax. Chest roentgenogram showed total left lung consolidation. On day 2 of her admission, the baby was endotracheally intubated due to left lung atelectasis complicating with respiratory distress. As bilious fluid discharge started to come out continuously from the endotracheal tube, the patient was submitted to multi-detector computed tomographic scan (MDCT) and Tc-99m hepatobiliary iminodiacetic acid (HIDA) scintigraphy. After the MDCT axial and coronal images were reformatted (Fig 1), a virtual bronchoscopy was performed that demonstrated a fistula coursing from the left main bronchus to the left hepatic lobe (Fig 2). Hepatobiliary scintigraphy revealed radio-tracer runoff in the left lung and dissemination through the right lung without radionuclide passage to gastrointestinal system. Thus, the patient was taken to operation with the presumptive diagnosis of a left BBF. In the operation, the BBF tract opening was located at the entrance of the left main bronchus by bronchoscopy. Then, a right posterolateral thoracotomy was performed from the fourth intercostal space. The bronchobiliary fistula was divided and ligated at the level of the tracheal entrance with nonabsorbable sutures (5-0 Prolene [Ethicon, Edinburgh, United Kingdom]). Explorative laparotomy revealed absence of common bile duct with a normal looking gallbladder. Intraoperative cholangiography was performed, and biliary tract connection to the gastrointestinal system was not demonstrated. Instead, contrast material filled up the intrahepatic biliary system, thus confirming the absence of the common bile duct. Roux-en-Y cholecysto-jejunostomy was used to resolve the biliary obstruction. Although biliary drainage ceased, and left lung atelectasis recovered on the postoperative early period, bile stained sputum recurred on postoperative day 3. The patient once more went through MDCT, and a thoracic cyst over the diaphragm was demonstrated (Fig 3). Hepatobiliary scintigraphy showed radio-tracer collection in the left lung and normal biliary drainage to the gastrointestinal system. She underwent a re-thoracotomy through the sixth intercostal space that demonstrated previously divided fistula ends were apart without connection in between. Further exploration of the posterior mediastinum revealed a cyst sited next to the esophagus, communicating with the bronchial tree and extending from the esophageal hiatus through the tracheal bifurcation with dimensions of 25 × 40 mm. After the aspiration of bile and further dissection, the cyst was excised with the distal ligation site at the diaphragm.Fig 2Virtual bronchoscopy image shows origin of broncho-biliary fistulae (arrow) in left main bronchus.View Large Image Figure ViewerDownload (PPT)Fig 3Postoperative multi-detector computed tomographic scan shows the remnant sac (arrow).View Large Image Figure ViewerDownload (PPT) Recovery was uneventful and the patient was discharged on postoperative day 5. She is still doing well without any complications for a follow-up period of 9 months. Histopathologic examination of the resected specimens in both of the operations revealed that they were lined by ciliated pseudostratified epithelium surrounded by a smooth muscle layer. Also there was a cartilage islet surrounded by mucinous glands. Congenital tracheobiliary or BBF is an uncommon anomaly. Although the majority of these cases have been seen in neonates, age distribution may vary from infants to adults [1Tekant G.A. Joseph V.T. Cheah S.L. Congenital tracheobiliary fistula.J Pediatr Surg. 1994; 29: 594-595Abstract Full Text PDF PubMed Scopus (17) Google Scholar, 2Egrari S. Krishnamoorthy M. Yee C.A. Applebaum H. Congenital bronchobiliary fistula: diagnosis and postoperative surveillance with HIDA scan.J Pediatr Surg. 1996; 31: 785-786Abstract Full Text PDF PubMed Scopus (27) Google Scholar, 3Kalayoglu M. Olcay I. Congenital bronchobiliary fistula associated with esophageal atresia and tracheo-esophageal fistula.J Pediatr Surg. 1976; 11: 463-464Abstract Full Text PDF PubMed Scopus (33) Google Scholar, 4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar, 5Aguilar C. Cano R. Camasca A. et al.Congenital bronchobiliary fistula detected by cholescintigraphy.Rev Gastroenterol Peru. 2005; 25: 216-218PubMed Google Scholar, 6Bringas Bollada M. Cabezas Martín M.H. Martínez Sagasti F. Ortuño Andériz F. Congenital bronchobiliary fistula diagnosed in adult age.Med Intensiva. 2006; 30: 475-476Crossref PubMed Scopus (7) Google Scholar, 7Levasseur P. Navajas M. Congenital tracheobiliary fistula.Ann Thorac Surg. 1987; 44: 318-319Abstract Full Text PDF PubMed Scopus (9) Google Scholar]. Various localizations of BBF have been described with only two on the left side [4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar]. Bilious vomiting, which is frequently misdiagnosed as gastroesophageal reflux, and respiratory distress are the most frequent symptoms in newborns [1Tekant G.A. Joseph V.T. Cheah S.L. Congenital tracheobiliary fistula.J Pediatr Surg. 1994; 29: 594-595Abstract Full Text PDF PubMed Scopus (17) Google Scholar, 2Egrari S. Krishnamoorthy M. Yee C.A. Applebaum H. Congenital bronchobiliary fistula: diagnosis and postoperative surveillance with HIDA scan.J Pediatr Surg. 1996; 31: 785-786Abstract Full Text PDF PubMed Scopus (27) Google Scholar, 4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar]. In the presented case, a high amount of clear bilious discharge from the endotracheal tube was the key symptom. Bilious vomiting in the absence of intestinal obstruction should be indicative for the anomaly [4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar]. Two embryological explanations have been advanced for the origin of the BBF. Neuhauser and colleagues [4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar], the first authors to describe this malformation in 1952, believed that the anomaly resulted from a duplication of the upper gastrointestinal tract. In 1957, Bremer [4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar], and then in 1966, Sane and colleagues [1Tekant G.A. Joseph V.T. Cheah S.L. Congenital tracheobiliary fistula.J Pediatr Surg. 1994; 29: 594-595Abstract Full Text PDF PubMed Scopus (17) Google Scholar, 4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar], suggested that presence of a communication between an anomalous bronchial bud and an anomalous bile duct would be explanatory. It is evident, as in our case, that the histologic features of the reported fistulas in the literature support the second theory [4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar]. Diagnostic studies are important for the surgery plan. In the present case, MDCT and virtual bronchoscopy demonstrated the anatomy of the anomaly. Hepatobiliary scintigraphy revealed biliary drainage to the tracheobronchial tree, as well as lack of connection between the biliary tract and the gastrointestinal system, which was also a clue for the anomaly. Because common bile duct atresia is the most common associated anomaly [1Tekant G.A. Joseph V.T. Cheah S.L. Congenital tracheobiliary fistula.J Pediatr Surg. 1994; 29: 594-595Abstract Full Text PDF PubMed Scopus (17) Google Scholar, 4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar, 6Bringas Bollada M. Cabezas Martín M.H. Martínez Sagasti F. Ortuño Andériz F. Congenital bronchobiliary fistula diagnosed in adult age.Med Intensiva. 2006; 30: 475-476Crossref PubMed Scopus (7) Google Scholar], we believe that performance of hepatobiliary scintigraphy is crucial for defining the accompanying biliary tract malformations [2Egrari S. Krishnamoorthy M. Yee C.A. Applebaum H. Congenital bronchobiliary fistula: diagnosis and postoperative surveillance with HIDA scan.J Pediatr Surg. 1996; 31: 785-786Abstract Full Text PDF PubMed Scopus (27) Google Scholar, 4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar]. Although intraoperative bronchoscopy demonstrated the fistula opening, we believe bile staining of the tracheobronchial tree could make this difficult. For this particular reason, performance of virtual bronchoscopy seems to be beneficial in showing the anatomy in better details compared with conventional bronchoscopy. We performed thoracotomy from the fourth intercostal space in the first operation to stay close to bronchial opening. Despite the ligation of the BBF, symptoms recurred and further radiological evaluation and reoperation were required. We assume that this condition was due to transitional obliteration of bile outlet leading to pressure increase in biliary tract causing postoperative opening of a fistula beyond the ligation place, which was not apparent in the first operation. This fistula could not be demonstrated in the intraoperative cholangiography, as it was not patent. Cessation of bilious discharge after the first operation might be suggestive for our hypothesis. On the other hand, it is possible that the cyst was present and missed in the first MDCT. As the computed tomographic images were taken in a little oblique manner, the cyst might partially overlay the density of the left lung leading to insufficient interpretation. Another possibility is that the lesion was missed at the first operation due to inadequate exposure. Therefore, we strongly recommend thoracotomy from the sixth intercostal space, which provides better exposure. Dissection of the BBF all the way down through the diaphragmatic hiatus and total excision is required rather than division and ligation of the fistula. As the fistula is the only bile outlet after BBF excision, biliary tract connection to the gastrointestinal system should be established. Roux-en-Y choledocho-jejunostomy can be performed for this purpose [4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar]. A fistulo-enteric anastomosis has also been described, and potentially could have been done at the region of the diaphragm [4Tommasoni N. Gamba P.G. Midrio P. Guglielmi M. Congenital tracheobiliary fistula.Pediatr Pulmonol. 2000; 30: 149-152Crossref PubMed Scopus (27) Google Scholar]. Porto-enterostomy may be another choice in case of an accompanying extrahepatic biliary atresia. If normal gallbladder exists, drainage of bile through cholecysto-enteric anastomosis can also be performed [1Tekant G.A. Joseph V.T. Cheah S.L. Congenital tracheobiliary fistula.J Pediatr Surg. 1994; 29: 594-595Abstract Full Text PDF PubMed Scopus (17) Google Scholar]. In the present case, we think that performance of Roux-en-Y cholecysto-jejunostomy was technically easy and appropriate by shortening operation time. The intra-abdominal portion of the fistula was remained untouched, not to cause problems, as it is recommended [1Tekant G.A. Joseph V.T. Cheah S.L. Congenital tracheobiliary fistula.J Pediatr Surg. 1994; 29: 594-595Abstract Full Text PDF PubMed Scopus (17) Google Scholar]. In conclusion, clear yellow-stained sputum or vomiting should alert the physician for the possibility of connection of the respiratory system and biliary tract. The MDCT and virtual bronchoscopy are the most important diagnostic tools for the detailed description of the anomaly. Hepatobiliary scintigraphy is crucial for defining the accompanying biliary tract malformations. An operation should be performed through the sixth intercostal space, and total removal of the BBF is mandatory.