Ann Thorac Surg 2006;82:330-332
© 2006 The Society of Thoracic Surgeons
Case report
Bilateral Communicating Bronchopulmonary Foregut Malformations in a Child
Arbinder Kumar Singal, MCh
a
,
*
,
V.R. Ravi Kumar, MCh
c
,
Madhusudana Rao, MCh
d
,
John Matthai, MD
b
a Department of Pediatric Surgery, PSG Institute of Medical Sciences, Coimbatore, Tamilnadu, India
b Department of Pediatrics, PSG Institute of Medical Sciences, Coimbatore, Tamilnadu, India
c Department of Pediatric Surgery, GKNM Hospital, Coimbatore, Tamilnadu, India
d Department of Cardiothoracic Surgery, GKNM Hospital, Coimbatore, Tamilnadu, India
Accepted for publication August 29, 2005.
* Address correspondence to Dr V. R. Ravi Kumar, 66 Co-operative Colony, K K Pudur, Coimbatore, Tamilnadu, 641038 India (Email: hemuravi{at}vsnl.com).
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Abstract
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Communicating bronchopulmonary foregut malformations are rare anomalies. The complex anatomy requires innovative surgical techniques. We report a child with bilateral sequestrations communicating with the lower esophagus. The sequestrations were excised through a single thoracotomy incision and the esophagus was repaired. Postoperatively the child has remained asymptomatic.
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Introduction
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Development of the foregut and its derivative (ie, the tracheobronchial tree) is a complex, but well orchestrated process. Disruption of this orderly sequence of events affects both the upper gastrointestinal tract as well as the airway giving rise to complex and surgically challenging malformations.
One such anomaly is the bronchopulmonary foregut malformation. The term was first introduced by Gerle and colleagues [1] in 1968 and was further elucidated by Clements and Warner [2]. Rarely these may present as communicating bronchopulmonary foregut malformations (ie, esophago-bronchial communication). We present a case with this complex anomaly that was successfully managed, and we review the relevant literature.
A 15-month-old female child was referred to our hospital with recurrent episodes of pneumonitis and failure to thrive. She also had a history of frequent regurgitation of feedings. On examination the child was pale and malnourished. Air entry was decreased in the left lower chest. Abdominal and cardiovascular system examination was essentially normal. A diagnosis of severe gastroesophageal reflux was entertained; therefore a barium swallow was done. Interestingly the barium swallow showed two tubular structures originating from the lower esophagus and leading to intrathoracic entities bilaterally (Fig 1a). A provisional diagnosis of bilateral communicating sequestration was made and a contrast enhanced computed tomographic scan with oral contrast was performed to further elucidate the anatomy. The contrast enhanced computed tomographic scan showed esophageal bronchi originating from the lower esophagus on both sides and communicating with intrathoracic sequestrations. The lesion on the left side was much bigger than the one on the right (Fig 1b).
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Fig 1. (a) Barium esophagogram showing esophageal bronchi on either side. (b) Contrast enhanced computed tomographic scan with oral contrast shows bronchi originating from the lower esophagus and intrathoracic sequestrations. Note that the right-sided lesion is comparatively smaller.
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The surgical plan included excision of bilateral sequestration, repair of the esophagus, and possibly an antireflux procedure and a feeding jejunostomy. A left thoracotomy was performed; the findings included left lower lobe intralobar sequestration communicating with the esophagus and a noncommunicating extralobar sequestration lying over the dome of left hemidiaphragm, both on the left side. The lower lobe had a dual blood supply; the basilar sequestred segments were receiving blood from a thick artery coming from the abdomen across the diaphragm, and the superior segment was coming from the pulmonary artery. The venous drainage was into the inferior pulmonary vein. The thick cartilaginous bronchus connecting the sequestration on the left side to the esophagus was dissected out and directly opposite it a bronchial communication was seen extending to the right side. A left lower lobectomy was performed; the abdominal artery and the esophageal communication were divided and the left lower lobe was removed. The right sequestration, seen to be quite small, was delivered into the left hemithorax from across the mediastinum. It was separated from the lower lobe, which was repaired. The blood supply (ie, a twig from the abdominal artery supplying the left lower lobe) was divided and the esophageal communication was severed. The esophageal wall was freshened and all the palpable cartilaginous tissue was removed (Fig 2a). The esophagus was closed in a linear fashion and the diaphragmatic hiatus was tightened around the esophagus. Intercostal drains were placed and a feeding jejunostomy was performed.
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Fig 2. (a) Resected specimens: the right-sided sequestration, center cartilage on the esophageal wall, and left lower lobe. (b) Postoperative barium study showing mild narrowing of the lower esophagus.
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A postoperative chest roentgenogram showed well expanded lungs. A contrast study on postoperative day 8 showed good flow into the stomach with no leakage. There was slight narrowing just above the gastroesophageal junction. The child tolerated oral feedings well, and a follow-up contrast study at 6 weeks showed similar findings (Fig 2b).
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Comment
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Bronchopulmonary foregut malformations include a wide variety of malformations such as pulmonary sequestrations, foregut duplication cysts, and diverticula of the gastrointestinal or pulmonary tree [1, 2]. Among these sequestrations are the most diverse and difficult to diagnose and treat. Pryce [3] coined the term sequestration in 1946 to describe an anatomically separate pulmonary mass that does not communicate with the normal airway and has an anomalous blood supply. Pulmonary sequestrations can be further divided into the extralobar type (in which the lesion is surrounded by a separate pleural sac) and the intralobar type (in which the pleura is shared with the native lung tissue).
Communicating bronchopulmonary foregut malformations are rare anomalies characterized by a pulmonary sequestration (extralobar type or intralobar type) with an aberrant bronchial connection to the foregut, generally esophagus or stomach. Srikanth and colleagues [4] proposed a classification of communicating bronchopulmonary foregut malformations in 1992. The present case should be considered as group 3, which is characterized by isolated anatomic lung lobe or segment communicating with the foregut.
The cause of communicating bronchopulmonary foregut malformation is controversial and different theories have been proposed. Any abnormalities in the tracheoesophageal sulcus, septum, or budding of the esophagus and respiratory tract may explain the persistence of respiratory elements connected to the alimentary tract [1, 2, 4, 5]. Bilateral communicating bronchopulmonary foregut malformations are extremely rare and only a handful of cases have been reported [6, 7]. We believe that only one case coexists that involved both extralobar type and intralobar type in a bilateral communicating bronchopulmonary foregut malformation, as previously described by Bratu and colleagues [6]; they reported successful excision of bilateral communicating extralobar type and intralobar type through a bilateral thoracotomy of a child. However, the child had long-term morbidity due to associated malformations. Joy and Abraham [7] reported bilateral intralobar sequestrations that communicated with the stomach.
In the present case, there were no associated anomalies and the child was referred after she sustained recurrent pneumonitis. We were able to resect the lesions through a unilateral thoracotomy, thus avoiding the morbidity of bilateral thoracotomy in a child with poor pulmonary reserve and general condition. We would also like to emphasize that children with severe regurgitation of feedings should be investigated early to rule out such rare and correctable anomalies.
In conclusion, management of these complex malformations requires an individualized approach and an innovative surgical technique. In the absence of associated anomalies, the prognosis is excellent.
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References
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- Gerle RD, Jaretzki 3rd A, Ashley CA, Berne AS. Congenital bronchopulmonary-foregut malformationpulmonary sequestration communicating the gastrointestinal tract. N Engl J Med 1968;278:1413-1419.[Medline]
- Clements BS, Warner JO. Pulmonary sequestration and related congenital bronchopulmonary-vascular malformationsnomenclature and classification based on anatomical and embryological considerations. Thorax 1987;42:401-408.[Abstract/Free Full Text]
- Pryce DM. Lower accessory pulmonary artery with intralobar sequestration of lungreport of cases. J Pathol 1949;58:457-467.
- Srikanth MS, Ford EG, Stanley P, Mahour GH. Communicating bronchopulmonary foregut malformations. Classification and embryogenesis J Pediatr Surg 1992;27:732-736.[Medline]
- Leithiser Jr RE, Capitanio MA, Macpherson RI, Wood BP. Communicating bronchopulmonary foregut malformations Am J Roentgenol 1986;146:227-231.[Abstract/Free Full Text]
- Bratu I, Flageole H, Chen MF, Di Lorenzo M, Yazbeck S, Laberge JM. The multiple facets of pulmonary sequestration J Pediatr Surg 2001;36:784-790.[Medline]
- Joy GM, Abraham MK. Bilateral communicating intralobar pulmonary sequestration Indian Pediatr 2005;42:729-730.[Medline]
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Abstract
Introduction
