Asian Cardiovasc Thorac Ann 2002;10:76-77
© 2002 Asia Publishing EXchange Pte Ltd
Congenital Pulmonary Lymphangiectasia With Chylothorax
Lee Seock Yeol, MD,
Yang Sung Rin, MD,
Lee Kihl Rho, MD
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Department of Thoracic and Cardiovascular Surgery Soonchunhyang University Kumi Hospital Kumi, Kyungsangbuk-do, Korea
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Lee Seock Yeol, MD Tel: 82 41 570 2193 Fax: 82 41 575 9674 email: csdoctor{at}korea.com
Department of Thoracic and Cardiovascular Surgery, Soonchunhyang University Chunan Hospital, 23 Bongmyung-dong, Chunan, Chungcheongnam-do 330-721, Korea.
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ABSTRACT
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A 5-year-old boy with dyspnea was diagnosed through thoracentesis as having chylothorax. After conservative management failed, open thoracotomy was performed. Lung biopsy confirmed the diagnosis of congenital pulmonary lymphangiectasia. Chylothorax was successfully controlled by fibrin glue pleurodesis in this patient.
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INTRODUCTION
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Congenital pulmonary lymphangiectasia is a rare and usually fatal disorder characterized by pulmonary subpleural, interlobar, perivascular, and peribronchial lymphatic dilatation. Its diagnosis is confirmed by lung biopsy. There is no definitive treatment yet.
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CASE REPORT
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A 5-year-old boy with dyspnea was admitted to the pediatric department of our hospital. Two years ago, he was diagnosed through right-sided open lung biopsy at another hospital as suffering from congenital pulmonary lymphangiectasia. Chest radiography showed hydrothorax and pneumonia (Figure 1A
). After thoracentesis, chylo-thorax was confirmed, and the patient was transferred to the Department of Thoracic and Cardiovascular Surgery. Subsequently, left closed thoracostomy was performed and conservative treatment given, including fasting and total parenteral nutrition. However, conservative treatment failed, and open thoracotomy was performed. Leakage of chyle was seen on the mediastinal pleura above the aortic arch. The sections before and after the ruptured thoracic duct were ligated with 5-0 polypropylene, and biopsy of the lateral lingular segment was performed. On post-operative day 1, about 300 mL of chyle was drained, and fibrin glue was instilled through the chest tube. After 7 days of fasting, no chyle was drained, and oral feeding was started. The chest tube was removed on the 10th postoperative day. Histopathologic examination of lung tissue showed dilated spaces lined with endothelial cells in the interlobar septa and the visceral pleura (Figure 2). We diagnosed this case as congenital pulmonary lym-phangiectasia with chylothorax. The boy was discharged on the 13th postoperative day. At 6-month follow-up, there was no recurrence or development of pleural effusion in either lung (Figure 1B).
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Figure 1. (A) Chest radiograph showing left hydrothorax and pneumonia on admission. (B) There was no recurrence or development of hydrothorax in either lung at 6-month follow-up.
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Figure 2. Photomicrograph showing dilated lymphatics in the interlobar septa and visceral pleura (hematoxylin and eosin stain, original magnification x100).
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DISCUSSION
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Congenital pulmonary lymphangiectasia is a rare disorder first described in 1856.1 There is a male predominance, and the mortality rate in newborns who present with respiratory distress is close to 100%.1 Although most cases are isolated, the disorder occurring in siblings and in association with 46,XY/46,XX mosaicism, ichthyosis congenita, Noonan's syndrome, Turner's syndrome, Fryns syndrome, Down's syndrome, and other unnamed syndromes that suggest genetic predisposition has been described.2
Noonan and colleagues1 classified congenital pulmonary lymphangiectasia into 3 groups: group 1, generalized lymphangiectasia; group 2, pulmonary venous obstruction with secondary lymphangiectasia; and group 3, primary pulmonary lymphatic development anomaly. Our case fell into group 3, which represents a primary develop-mental defect of lung lymphatics that is isolated, without other heart or lymphatic abnormalities. The pathogenesis is unknown but is postulated to be due to a developmental error in which the normal regression of connective tissue elements fails to occur after the 16th week of fetal life.1 This group shows the highest perinatal mortality rate (29 of the 30 children described by Noonan's group1 died during infancy). Radiographic appearance may be in-distinguishable from pulmonary interstitial emphysema or chronic lung disease. Diagnosis is difficult to make as it requires lung biopsy in a patient who is typically unstable.1
Surgical indication for chylothorax has evolved with time. In 1973, Selle and colleagues3 advocated surgery when chylous outflow exceeded 1,500 mL/day and persisted for more than 14 days. In 1981, Patterson and colleagues4 suggested that intervention should be instituted after 7 days. Ulibarri and colleagues5 recommended surgery when chylous outflow continued for more than 5 days, to prevent metabolic and immunological complications. In the treatment of chylothorax, success with the use of fibrin glue has been reported. This method was first used by Stenzl and colleagues.6
Our case was easily confirmed as congenital pulmonary lymphangiectasia. Surgery and fibrin glue pleurodesis successfully controlled chylothorax in this patient.
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REFERENCES
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Noonan JA, Walters LR, Reeves JT. Congenital pulmonary lymphangiectasis. Am J Dis Child
1970;120:314–9.[Abstract/Free Full Text]
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Chung CJ, Fordham LA, Barker P, Cooper LL. Children with congenital pulmonary lymphangiectasia: after infancy. AJR Am J Roentgenol
1999;173:1583–8.[Abstract]
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Selle JG, Snyder WH III, Schreiber JT. Chylothorax: indications for surgery. Ann Surg
1973;177:245–9.[Medline]
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Patterson GA, Todd TR, Delarue NC, Ilves R, Pearson FG, Cooper JD. Supradiaphragmatic ligation of the thoracic duct in intractable chylous fistula. Ann Thorac Surg
1981; 32:44–9.[Abstract]
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Ulibarri JI, Sanz Y, Fuentes C, Mancha A, Aramendia M, Sanchez S. Reduction of lymphorrhagia from ruptured thoracic duct by somatostatin. Lancet
1990;336:258.
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Stenzl W, Rigler B, Tscheliessnigg KH, Beitzke A, Metzler H. Treatment of postsurgical chylothorax with fibrin glue. Thorac Cardiovasc Surg
1983;31:35–6.[Medline]
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ABSTRACT
INTRODUCTION
