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Ann Thorac Surg 2004;77:695-697
© 2004 The Society of Thoracic Surgeons

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Case report

Pediatric thoracic lymphangiomatosis: is chest wall resection too radical?

^a Department of Thoracic Surgery, University Hospital Leuven, Leuven, Belgium
^b Department of Paediatrics, University Hospital Leuven, Leuven, Belgium

Accepted for publication May 12, 2003.

^* Address reprint requests to Dr Papagiannopoulos, Department of Cardiothoracic Surgery, Leeds General Infirmary, Jubilee Building, Level D, Great George St, Leeds LS1 3EX, UK
e-mail: kpapagiannopoulos{at}yahoo.com

	Abstract

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Lymphangiomas account for 5% to 6% of all pediatric neoplasms. Complex pathologic processes often present a diagnostic and treatment challenge. Radical and major surgery might have a role in complex cases when medical treatment fails.

	Introduction

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The management of pediatric lymphangiomatosis is challenging, and recurrences have been reported even after surgical intervention. Thoracic lymphangiomatosis often presents with chylothoraces with nutritional and immunologic sequelae. We report the case of a pediatric patient with thoracic lymphangiomatosis treated with extensive chest wall resection and reconstruction.

A 4-year-old boy was referred to our department by the pediatric pulmonologists. He was born in March 1996; the delivery was not complicated. He thrived until the age of 6 months when episodes of severe bronchospasm developed. The diagnosis was asthma. The chest roentgenograms were reported to be normal.

At the age of 1 year, the infant was readmitted because of acute dyspnea and anemia. The chest roentgenogram showed a large pleural effusion, which was drained with thoracentesis, and a fractured sixth rib (Fig 1). We could not retrieve the results of the fluid biochemical analysis. A rib biopsy was performed to exclude malignancy but resulted in no definitive diagnosis.

View larger version (102K): [in this window] [in a new window]   Fig 1. Chest roentgenogram showing fractured sixth rib and abnormalities in the fourth to eighth ribs.

Lymphoscintigraphy revealed diffuse uptake in the right hemithorax with no specific lymphoid lakes and a normal thoracic duct. The chest tomogram and magnetic resonance imaging scan confirmed the abnormalities seen on the chest roentgenogram and showed no evidence of other intrathoracic or extrathoracic pathologic processes.

Initial management with a medium-chain triglyceride diet and multiple aspirations failed to resolve the problem. In August, treatment with interferon- was initiated but was discontinued because of side effects. The chylous leak increased, and a passive pleuroperitoneal shunt was inserted. It required five revisions within 6 months owing to dysfunction. Total parenteral nutrition was started, and the patient was transferred to our unit.

Initially a tube thoracostomy was performed to lessen the symptoms. We noted a chylous leak of approximately 80 to 100 mL/h. We finally decided to operate in view of the massive production of chyle, the extensive abnormality in the chest wall, and the failure of other treatment modalities.

At thoracotomy, inspection of the chest wall revealed ribs 3 to 8 to be small, multiply notched, and replaced by fibrous bands in areas. A moderate residual effusion was present. Chyle was "sweating" diffusely from the chest wall at a rate of 100 to 120 mL/h even when the thoracic duct was ligated. A chest wall resection including all involved ribs and soft tissues was performed. The large defect was replaced with a sandwich of Marlex mesh reinforced with methylmethacrylate to provide support for the scapula and good cosmetic results. It was covered completely by the spared latissimus dorsi and serratus anterior muscles. A single drain was left in situ.

Macroscopically, the resected specimen consisted of full-thickness chest wall, measured 16 x 9 x 1 cm, and contained six ribs and soft tissue. All ribs were extremely friable with multiple notchings and areas of cystic degeneration. Microscopically, multiple lesions were present in all chest wall elements. The lesions were of variable size and comprised of irregular vessels lined mainly by flat endothelium and embedded in a collagen stroma with evident lymphoid cells and aggregates. The histologic picture was compatible with lymphangiomatosis. There was no evidence of malignancy.

The postoperative course was uncomplicated. The patient was extubated 3 days after operation, and the drain was removed 48 hours after he had begun normal oral feedings. His last appointment was 2.5 years after operation. There was no recurrence of the effusion, but scoliosis had begun to develop.

	Comment

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Lymphangiomas account for 5% to 6% of all pediatric neoplasms [1]. Those involving bones are extremely rare and often multiple [2]. The presentation varies from asymptomatic lesions found on chest roentgenograms to pathologic fractures, neurologic dysfunction, and severe respiratory compromise as a result of large chylous collections [3]. Progressive lysis of bones and their replacement with fibrous strands (Gorham's disease) has been reported [2, 4].

Diagnosis is often made on the basis of the presence of lytic bone lesions with a characteristic "soap bubble" appearance. Lymphangiograms are essential to identify other lesions and should always be done before operation. Reports have shown that bone scans are not always as sensitive as T2-weighted magnetic resonance imaging scans, which can reveal unsuspected lesions elsewhere and thus alter the treatment planning [5, 6].

Several treatments have been reported, though rates of success and recurrence vary. A medium-chain triglyceride diet is not useful, and total parenteral nutrition should be started initially. Thoracentesis or tube thoracostomy can improve symptoms temporarily. The use of sclerosing agents (glucose 50%, tetracycline, OKT-432, bleomycin sulfate talc) produces variable success, and recurrences have been reported. Interferon- has been used with limited positive results. Radiotherapy has been successful in a few instances. However, there is still no agreement as to the best radiation doses to treat this disease [7, 8].

The classic surgical procedures involve pleurodesis, ligation of the thoracic duct, and pleuroperitoneal shunts [7, 9]. We have not found chest wall resection reported in pediatric patients. In our patient, resection of the involved chest wall resolved the problem. Initial ligation of the thoracic duct did not arrest the chylous leak. In extensive forms of thoracic lymphangiomatosis, the thoracic duct might not be the responsible vessel but rather the extensive network of abnormal lymphatic vessels [4]. There still remains the question whether such extensive resection is appropriate in growing patients. Long-term follow-up as well as experiences from other institutions is necessary to determine its effectiveness [6].

	References

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1. Faul J.L., Berry G.J., Colby T.V., et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000;161(3 Pt 1):1037-1046.[Free Full Text]
2. Podevin G., Levard G., Larroquet M., Gruner M. Pleuroperitoneal shunt in the management of chylothorax caused by thoracic lymphatic dysplasia. J Pediatr Surg 1999;34:1420-1422.[Medline]
3. Johnson D.W., Klazynski P.T., Gordon W.H., Russell D.A. Mediastinal lymphangioma and chylothorax: the role of radiotherapy. Ann Thorac Surg 1986;41:325-328.[Abstract/Free Full Text]
4. Murphy M.C., Newman B.M., Rodgers B.M. Pleuroperitoneal shunts in the management of persistent chylothorax. Ann Thorac Surg 1989;48:195-200.[Abstract/Free Full Text]
5. Konez O., Vyas P.K., Goyal M. Disseminated lymphangiomatosis presenting with massive chylothorax. Pediatr Radiol 2000;30:35-37.[Medline]
6. Alqahtani A., Nguyen L.T., Hageola H., Shaw K., Laberge J.M. 25 Years' experience with lymphangiomas in children. J Pediatr Surg 1999;34:1164-1168.[Medline]
7. Tucker S.M. Bilateral chylothorax with multiple osteolytic lesions? Generalised abnormality of lymphatic system. Proc R Soc Med 1967;60:17-19.[Medline]
8. Rostrom A.Y. Treatment of thoracic lymphangiomatosis. Arch Dis Child 2000;83:138-139.[Free Full Text]
9. Canil K., Fitzgerald P., Lau G. Massive chylothorax associated with lymphangiomatosis of the bone. J Pediatr Surg 1994;29:1186-1188.[Medline]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Dirk E. M. Van Raemdonck Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Papagiannopoulos, K. Articles by Lerut, T. Search for Related Content PubMed PubMed Citation Articles by Papagiannopoulos, K. Articles by Lerut, T. Related Collections Chest wall