Ann Thorac Surg 2005;79:1099
© 2005 The Society of Thoracic Surgeons
Correspondence
Lymphangiomatosis Possibly With Gorham's Disease and Not Lymphangioma
Sameh Ibrahim Sersar, MBBCH
Mansoura University, Faculty of MedicineElgomhoria St 123Mansoura, Egypt
sameh001{at}yahoo.com
To the Editor:
We read with great interest the case report concerning pediatric thoracic lymphangiomatosis and the role of chest wall resection by Papagiannopoulos and associates [1]. Disorders of the pulmonary lymphatic system range from trauma, cancer, lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Because these disorders are rare and infiltrative, they are often misdiagnosed, and management is difficult [2, 3].
We believe the condition described in the case report is lymphangiomatosis possibly associated with Gorham's disease and not lymphangioma. There are differences between the two entities. Lymphangiomatosis usually presents in childhood without sex predilection and occurs wherever lymphatics are found, but most often in the thorax and neck. The disease process, in a single or multiple form, can be found within the mediastinum, adherent to the pleura, or within the chest wall. Mediastinal fat can be diffusely infiltrated with anastomosing dilated lymphatics. Chylothorax, chyloptysis, hemoptysis, chylopericardium, chylous ascites, protein wasting enteropathy, peripheral lymphedema, hemihypertrophy, lymphopenia, and disseminated intravascular coagulopathy have been described.
When the patient in the case report by Papagiannopoulos and coauthors was 1 year old, the chest roentgenogram showed a large pleural effusion, which was drained by thoracentesis, and a fractured sixth rib. The authors could not retrieve the results of the biochemical analysis. We would have preferred tube thoracostomy instead of aspiration because of the child's age and the nature of the fluid (chyle). A rib biopsy was done to exclude malignancy but produced no definitive diagnosis. We understood that this rib was excised, as we know that the least chest wall biopsy was an excision biopsy.
Several years later, the patient was admitted again. Biochemical analysis of the pleural fluid showed chyle, and the diagnosis was thoracic lymphangiomatosis. The authors aspirated again. Why did they not insert a tube? We believe this patient did not receive the best treatment options possible. He had repeat reaspirations and five pleuroperitoneal shunts, which are palliative and will complicate further procedures. When it was decided to do a thoracotomy, the authors did not perform a parietal pleurectomy, which might have solved the problem. We do not see any place for the use of interferon-
in such a young patient. What about the use of etilefrine hydrochloride for the management of chylous leaks [4]? We think that chest wall excision and reconstruction was not a wise procedure for this patient. We prefer earlier thoracic duct ligation together with parietal pleurectomy or pleurodesis with radiotherapy or both measures. Chest wall reconstruction in such a case is just the start of a lot of complications: impairment of growth, foreign-body reaction, carcinogenesis, or a combination of these. Scoliosis was already apparent. In such children, we must consider Noonan's syndrome, asplenia, Gorham's disease and tuberous sclerosis.
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References
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- Papagiannopoulos K, Van Raemdonck DEM, De Boeck K, Lerut T. Pediatric thoracic lymphangiomatosis: is chest wall resection too radical? Ann Thorac Surg. 2004;77:695–697[Abstract/Free Full Text]
- Hilliard RI, McKendry JB, Phillips MJ. Congenital abnormalities of the lymphatic system: a new clinical classification. Pediatrics. 1990;86:988–994[Abstract/Free Full Text]
- Sameh IS, Ismaeil MF, Nasser MAF, ElSaeid AMM. Huge cervico-thoracic thymic cyst. Interactive Cardiovasc Thorac Surg. 2003;2:339–340[Abstract/Free Full Text]
- Guillem P, Papachristos I, Peillon C, Triboulet J-P. Etilefrine use in the management of post-operative chyle leaks in thoracic surgery. Interactive Cardiovasc Thorac Surg. 2004;3:156–160[Abstract/Free Full Text]
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