An Uncommon Mediastinal Cyst With a Rare Neurologic Consequence Sivakumar Sivalingam, FRCS, Ahmad U. Qureshi, FCPS, Leong M. Chern, MRCPCH, and Haifa A. Latiff, MMed Cardiothoracic Surgery Department and Paediatric Cardiology Department, National Heart Institute, Kuala Lumpur, Malaysia
Enteric cysts are uncommon posterior mediastinal cysts, usually presenting with respiratory symptoms in infancy. We present a rare case of posterior mediastinal enteric cyst extending from below the diaphragm and perforating into the left atrium, causing a thromboembolic cerebral infarct in a 5-year-old boy. (Ann Thorac Surg 2014;97:e93–5) Ó 2014 by The Society of Thoracic Surgeons
F
oregut cysts constitute 10% to 18% of all mediastinal masses in children. Bronchogenic cysts are by far the most common, occurring in up to 70% of cases. Enteric cysts are relatively rare malformations [1]. Also called enterogenic or enterogenous cysts, they account for 7% to 20% of posterior mediastinal cysts. Enteric cysts usually present with respiratory symptoms in infancy. We describe a rare case of posterior mediastinal enteric cyst extending from below the diaphragm, involving the posterior wall of the inferior vena cava (IVC) and the right atrium (RA), and perforating across the interatrial septum (IAS) into the left atrium (LA), causing a thromboembolic cerebral infarct in a 5-year-old boy. A 5-year-old boy, previously healthy and developmentally normal, was brought to our department with a history of low-grade fever for 2 weeks and a sudden onset of right hemiplegia secondary to a thromboembolic event involving the left middle cerebral artery territory. The results of a septic profile were negative, with borderline C-reactive protein levels (17 mg/L). An echocardiogram showed a large pedunculated nonhomogenous mobile mass in the LA with irregular margins attached to the IAS. A clot or vegetation was suspected, and the patient was given a heparin infusion and an empirical antibiotic agent for infective endocarditis. Despite 1 week of treatment, the mass did not regress in size. Considering the high risk for the development of further thromboembolic events, surgical intervention was performed. After bicaval cannulation, the area around the IVC appeared unusually thick, causing difficulty in passing a snare around the IVC. The LA was approached through the IAS with the patient under cardiopulmonary bypass. At the point of attachment of the mass to the septum, a sinus was noted Accepted for publication Dec 9, 2013. Address correspondence to Dr Qureshi, 348 Block B, Revenue Employees Cooperative Housing Society, Lahore, 54700, Pakistan; e-mail: [email protected].
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
and excised. The sinus was believed to be just a residual outpouching after resection of the mass and was not probed further at that time. Histopathologic findings showed a darkish pedunculated mass measuring 20 13 10 mm and composed of necrotic material, fibrin, some red blood cells, and fairly abundant leukocytes, mainly neutrophils. No viable tissue or tumor cells were seen. Gram staining for bacterial organisms, acid-fast bacilli staining and cultures showed no infective organism. In consideration of the abundance of leukocytes, antibiotics were continued to complete a 6-week course, with resolution of fever and normalization of acute phase reactants. The Hemiplegia improved to grade 3/5 hemiperisis. Studies for immune deficiency states and procoagulopathy also had negative results. The patient was brought in again after 4 months with a history of low-grade fever and recurrence of the LA mass in nearly the same dimensions (Fig 1). On further detailed workup, computed tomography of the thorax identified a cystic mass in the posterior mediastinum behind the heart chambers, but its extent and origin could not be established. The patient underwent an exploratory operation under cardiopulmonary bypass and hypothermic circulatory arrest. A thick-walled cystic structure was found extending, from the dome of the liver across the diaphragm, adjacent to the posterior wall of the IVC and the RA, opening through the IAS into the LA just above the annulus of the mitral valve. The lesion was excised along with the part of the diaphragm and the wall of the IVC to which it was adherent. The LA was opened along Sondergaard’s groove. From within, the cystic lesion was excised around its opening, including the IAS and the RA with its junction with the IVC (Fig 2A, B). The IAS was closed with direct closure with Prolene 5-0 sutures. The LA was closed with Prolene 5-0 sutures. The defect in the RA along with the IVC was closed with a patch of bovine pericardium by use of Prolene 6-0 sutures. The histopathologic feature of the cyst wall outside the LA showed a fibrotic wall, occasional smooth muscle tissue with areas of benign gastric antral type mucosal lining, focal ulceration, and reactive fibrovascular granulation tissue (Fig 2C). No malignant transformation was seen. The cyst wall from inside the LA showed a piece of fibrin clot consistent with thrombus. The diagnosis of posterior mediastinal gastroenteric cyst with perforation into the LA was made. The patient had an uneventful postoperative period and discharged on day 7. At the 5-year follow-up, there was no recurrence, and neurologically, hemiperisis had improved to a motor deficit of 4/5.
Comment The bronchial tree and the digestive tract arise from a common primitive foregut during embryogenesis. Endodermal in origin, the differentiation occurs by the fourth week of gestation, with the ventral bud giving rise to the bronchial tract and the dorsal bud developing into gastrointestinal tract. Foregut cysts are rare congenital malformations arising from the abnormal off-budding 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.12.060
e94
CASE REPORT SIVALINGAM ET AL COMPLICATED MEDIASTINAL ENTERIC CYST
Ann Thorac Surg 2014;97:e93–5
Fig 1. Transthoracic echocardiogram showing (A) a large pedunculated mass in the left atrium, (B) complete resection with no residual mass in the left atrium postoperatively that recurred (C) after the first resection.
from the primitive foregut between the third and seventh weeks of gestation. Many diverse theories have been proposed, including split notochord syndrome, persistence of neuroenteric band, foregut vacuole formation, and faulty separation of notochord and endoderm during fetal life. The exact mechanism is still unknown [2]. The definite diagnosis is based on histologic confirmation of columnar epithelium, additional gastric mucosa, smooth muscles or cartilage, and even pancreatic tissue [2]. Histologically, foregut cysts are classified as bronchogenic and enteric cysts, depending on the cyst wall lining. Enteric cysts are further labeled as neuroenteric or gastroenteric cysts, depending on additional nervous tissue or gastric mucosal cellularity. Pancreatic and thymic tissue has rarely been reported. A common nomenclature has not been adopted, and various interchangeable terms have been widely used in the literature to describe similar lesions [2]. Up to two thirds of children with foregut cysts become symptomatic in early life and present in infancy with compression symptoms on the airway or esophagus. Associated vertebral anomalies may complicate the symptoms, worsening the respiratory issues. In adult life, Fig 2. Operative findings. (A) Mass protruding from the posterior wall of the left atrium through the interatrial septum. (B) Mass measured 2 1.3 cm. (C) Histologic evidence of single columnar enteric epithelium (arrowhead) with areas of squamous metaplasia (arrow); occasional areas of gastric mucosa were also identified (hematoxylin and eosin stain, 10).
the cysts present with nonspecific chest pain or epigastric discomfort. The cysts are prone to become infected [3]. There have been reports of malignant transformation and adenocarcinoma developing in the cyst wall. In severe cases, the gastric musoca may develop ulceration and perforate into the surrounding structures. The patients usually present with hemoptysis or hematemesis, depending on the site of perforation [4]. Bronchogenic cysts have been reported to involve the IAS, the RA, and even the left ventricular outflow tract [5, 6]. There has been one report of perforation of an enteric cyst into the pericardium, causing tamponade [4]. Our case is the first documented case of gastroenteric cyst involving the RA and the IAS with perforation into the LA, presenting with a neurologic symptom from subsequent thromboembolism. These cysts do not undergo spontaneous regression [3]. Resection of asymptomatic cysts is indicated because of the high incidence of infection, ulceration, perforation, and even malignant transformation [7, 8]. Thoracoscopic resection has been possible in small isolated cysts. In cysts having a common wall with surrounding structures, wide radical resection is indicated. As in our case, there has been no published report of recurrence after complete resection [3].
Ann Thorac Surg 2014;97:e93–5
The authors thank Dr Pathmanathan Rajadurai for histopathologic review of the specimen.
References 1. Reynolds M. Foregut cysts of the mediastinum in infants and children. In: Shields TW, LoCicero J III, Ponn RB, eds. General Thoracic Surgery. Vol 2. 5th ed. Philadelphia: Williams & Wilkins; 2000: pp. 2393–9. 2. Sharma S, Nezakatgoo N, Sreenivasan P, Vanatta J, Jabbour N. Foregut cystic developmental malformation: new taxonomy and classification—unifying embryopathological concepts. Indian J Pathol Microbiol 2009;52:461–72.
CASE REPORT SIVALINGAM ET AL COMPLICATED MEDIASTINAL ENTERIC CYST
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3. Juhasz A, Botos B, Sark any A, Szontagh-Kishazi P, Varga I, Altorjay A. Considerations in the surgical treatment of enterogenic mediastinal cyst. Orv Hetil 2005;146:2417–9. 4. Sentís MI, Sanchís JB, Garolera JMG, Biel MC, Garay MR, Ruiz JR. Mediastinal enteric cyst: unusual clinical presentation and histopathology. Arch Bronconeumol 2004;40:185–7. 5. Huang JH, Rudzinski ER, Minette MS, Langley SM. First case of intracardiac foregut cyst occurring in the left-ventricular outflow tract. Pediatr Cardiol 2013;34:2060–2. 6. Curran R, Paterson HS. Surgical treatment of intramyocardial foregut cysts. Ann Thorac Surg 2005;80:737–9. 7. Dubrava J, Koren J, Pospisilova V. Mediastinal foregut duplication cyst of enteric type containing a persistent thymus, imitating a pericardial cyst. Bratisl Lek Listy 2013;114:480–3. 8. Suzuki K, Koyama S, Yamada S, Kawabata Y. Adenocarcinoma arising in a mediastinal enteric cyst. Intern Med 2007;46:781–4.
Enteric cysts are uncommon posterior mediastinal cysts, usually presenting with respiratory symptoms in infancy. We present a rare case of posterior mediastinal enteric cyst extending from below the diaphragm and perforating into the left atrium, causing a thromboembolic cerebral infarct in a 5-year-old boy. (Ann Thorac Surg 2014;97:e93–5) Ó 2014 by The Society of Thoracic Surgeons
F
oregut cysts constitute 10% to 18% of all mediastinal masses in children. Bronchogenic cysts are by far the most common, occurring in up to 70% of cases. Enteric cysts are relatively rare malformations [1]. Also called enterogenic or enterogenous cysts, they account for 7% to 20% of posterior mediastinal cysts. Enteric cysts usually present with respiratory symptoms in infancy. We describe a rare case of posterior mediastinal enteric cyst extending from below the diaphragm, involving the posterior wall of the inferior vena cava (IVC) and the right atrium (RA), and perforating across the interatrial septum (IAS) into the left atrium (LA), causing a thromboembolic cerebral infarct in a 5-year-old boy. A 5-year-old boy, previously healthy and developmentally normal, was brought to our department with a history of low-grade fever for 2 weeks and a sudden onset of right hemiplegia secondary to a thromboembolic event involving the left middle cerebral artery territory. The results of a septic profile were negative, with borderline C-reactive protein levels (17 mg/L). An echocardiogram showed a large pedunculated nonhomogenous mobile mass in the LA with irregular margins attached to the IAS. A clot or vegetation was suspected, and the patient was given a heparin infusion and an empirical antibiotic agent for infective endocarditis. Despite 1 week of treatment, the mass did not regress in size. Considering the high risk for the development of further thromboembolic events, surgical intervention was performed. After bicaval cannulation, the area around the IVC appeared unusually thick, causing difficulty in passing a snare around the IVC. The LA was approached through the IAS with the patient under cardiopulmonary bypass. At the point of attachment of the mass to the septum, a sinus was noted Accepted for publication Dec 9, 2013. Address correspondence to Dr Qureshi, 348 Block B, Revenue Employees Cooperative Housing Society, Lahore, 54700, Pakistan; e-mail: [email protected].
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
and excised. The sinus was believed to be just a residual outpouching after resection of the mass and was not probed further at that time. Histopathologic findings showed a darkish pedunculated mass measuring 20 13 10 mm and composed of necrotic material, fibrin, some red blood cells, and fairly abundant leukocytes, mainly neutrophils. No viable tissue or tumor cells were seen. Gram staining for bacterial organisms, acid-fast bacilli staining and cultures showed no infective organism. In consideration of the abundance of leukocytes, antibiotics were continued to complete a 6-week course, with resolution of fever and normalization of acute phase reactants. The Hemiplegia improved to grade 3/5 hemiperisis. Studies for immune deficiency states and procoagulopathy also had negative results. The patient was brought in again after 4 months with a history of low-grade fever and recurrence of the LA mass in nearly the same dimensions (Fig 1). On further detailed workup, computed tomography of the thorax identified a cystic mass in the posterior mediastinum behind the heart chambers, but its extent and origin could not be established. The patient underwent an exploratory operation under cardiopulmonary bypass and hypothermic circulatory arrest. A thick-walled cystic structure was found extending, from the dome of the liver across the diaphragm, adjacent to the posterior wall of the IVC and the RA, opening through the IAS into the LA just above the annulus of the mitral valve. The lesion was excised along with the part of the diaphragm and the wall of the IVC to which it was adherent. The LA was opened along Sondergaard’s groove. From within, the cystic lesion was excised around its opening, including the IAS and the RA with its junction with the IVC (Fig 2A, B). The IAS was closed with direct closure with Prolene 5-0 sutures. The LA was closed with Prolene 5-0 sutures. The defect in the RA along with the IVC was closed with a patch of bovine pericardium by use of Prolene 6-0 sutures. The histopathologic feature of the cyst wall outside the LA showed a fibrotic wall, occasional smooth muscle tissue with areas of benign gastric antral type mucosal lining, focal ulceration, and reactive fibrovascular granulation tissue (Fig 2C). No malignant transformation was seen. The cyst wall from inside the LA showed a piece of fibrin clot consistent with thrombus. The diagnosis of posterior mediastinal gastroenteric cyst with perforation into the LA was made. The patient had an uneventful postoperative period and discharged on day 7. At the 5-year follow-up, there was no recurrence, and neurologically, hemiperisis had improved to a motor deficit of 4/5.
Comment The bronchial tree and the digestive tract arise from a common primitive foregut during embryogenesis. Endodermal in origin, the differentiation occurs by the fourth week of gestation, with the ventral bud giving rise to the bronchial tract and the dorsal bud developing into gastrointestinal tract. Foregut cysts are rare congenital malformations arising from the abnormal off-budding 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.12.060
e94
CASE REPORT SIVALINGAM ET AL COMPLICATED MEDIASTINAL ENTERIC CYST
Ann Thorac Surg 2014;97:e93–5
Fig 1. Transthoracic echocardiogram showing (A) a large pedunculated mass in the left atrium, (B) complete resection with no residual mass in the left atrium postoperatively that recurred (C) after the first resection.
from the primitive foregut between the third and seventh weeks of gestation. Many diverse theories have been proposed, including split notochord syndrome, persistence of neuroenteric band, foregut vacuole formation, and faulty separation of notochord and endoderm during fetal life. The exact mechanism is still unknown [2]. The definite diagnosis is based on histologic confirmation of columnar epithelium, additional gastric mucosa, smooth muscles or cartilage, and even pancreatic tissue [2]. Histologically, foregut cysts are classified as bronchogenic and enteric cysts, depending on the cyst wall lining. Enteric cysts are further labeled as neuroenteric or gastroenteric cysts, depending on additional nervous tissue or gastric mucosal cellularity. Pancreatic and thymic tissue has rarely been reported. A common nomenclature has not been adopted, and various interchangeable terms have been widely used in the literature to describe similar lesions [2]. Up to two thirds of children with foregut cysts become symptomatic in early life and present in infancy with compression symptoms on the airway or esophagus. Associated vertebral anomalies may complicate the symptoms, worsening the respiratory issues. In adult life, Fig 2. Operative findings. (A) Mass protruding from the posterior wall of the left atrium through the interatrial septum. (B) Mass measured 2 1.3 cm. (C) Histologic evidence of single columnar enteric epithelium (arrowhead) with areas of squamous metaplasia (arrow); occasional areas of gastric mucosa were also identified (hematoxylin and eosin stain, 10).
the cysts present with nonspecific chest pain or epigastric discomfort. The cysts are prone to become infected [3]. There have been reports of malignant transformation and adenocarcinoma developing in the cyst wall. In severe cases, the gastric musoca may develop ulceration and perforate into the surrounding structures. The patients usually present with hemoptysis or hematemesis, depending on the site of perforation [4]. Bronchogenic cysts have been reported to involve the IAS, the RA, and even the left ventricular outflow tract [5, 6]. There has been one report of perforation of an enteric cyst into the pericardium, causing tamponade [4]. Our case is the first documented case of gastroenteric cyst involving the RA and the IAS with perforation into the LA, presenting with a neurologic symptom from subsequent thromboembolism. These cysts do not undergo spontaneous regression [3]. Resection of asymptomatic cysts is indicated because of the high incidence of infection, ulceration, perforation, and even malignant transformation [7, 8]. Thoracoscopic resection has been possible in small isolated cysts. In cysts having a common wall with surrounding structures, wide radical resection is indicated. As in our case, there has been no published report of recurrence after complete resection [3].
Ann Thorac Surg 2014;97:e93–5
The authors thank Dr Pathmanathan Rajadurai for histopathologic review of the specimen.
References 1. Reynolds M. Foregut cysts of the mediastinum in infants and children. In: Shields TW, LoCicero J III, Ponn RB, eds. General Thoracic Surgery. Vol 2. 5th ed. Philadelphia: Williams & Wilkins; 2000: pp. 2393–9. 2. Sharma S, Nezakatgoo N, Sreenivasan P, Vanatta J, Jabbour N. Foregut cystic developmental malformation: new taxonomy and classification—unifying embryopathological concepts. Indian J Pathol Microbiol 2009;52:461–72.
CASE REPORT SIVALINGAM ET AL COMPLICATED MEDIASTINAL ENTERIC CYST
e95
3. Juhasz A, Botos B, Sark any A, Szontagh-Kishazi P, Varga I, Altorjay A. Considerations in the surgical treatment of enterogenic mediastinal cyst. Orv Hetil 2005;146:2417–9. 4. Sentís MI, Sanchís JB, Garolera JMG, Biel MC, Garay MR, Ruiz JR. Mediastinal enteric cyst: unusual clinical presentation and histopathology. Arch Bronconeumol 2004;40:185–7. 5. Huang JH, Rudzinski ER, Minette MS, Langley SM. First case of intracardiac foregut cyst occurring in the left-ventricular outflow tract. Pediatr Cardiol 2013;34:2060–2. 6. Curran R, Paterson HS. Surgical treatment of intramyocardial foregut cysts. Ann Thorac Surg 2005;80:737–9. 7. Dubrava J, Koren J, Pospisilova V. Mediastinal foregut duplication cyst of enteric type containing a persistent thymus, imitating a pericardial cyst. Bratisl Lek Listy 2013;114:480–3. 8. Suzuki K, Koyama S, Yamada S, Kawabata Y. Adenocarcinoma arising in a mediastinal enteric cyst. Intern Med 2007;46:781–4.
![[A rare cause of mediastinal cyst].](/assets/img/hold.png)
