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Recurrent Mixed Capillary Cavernous Lymphangioma: A Unique Cause for Recurrent Acute Respiratory Failure FREE TO VIEW

Omar Mousa, MD; Vanessa Hoy, MD; Andreea Nitu-Marquise, MD; Matthew Hess, MD
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SUNY Upstate, Syracuse, NY

Chest. 2014;146(4_MeetingAbstracts):299A. doi:10.1378/chest.1994092
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SESSION TITLE: Critical Care Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Lymphangiomas are malformations of the lymphatic system. They are classified into cystic being the most common, capillary, and cavernous types. Most lymphangiomas are diagnosed by age 2. They occur 75% in the head and neck, 25% in the axilla, and other areas such as the mediastinum (1%) are extremely rare. Though benign, larger lesions imposing on surrounding organs increases the morbidity. Here we present a rare case of a left chest wall recurrent mixed capillary cavernous lymphangioma (MCCL) causing frequent acute on chronic hypercapnic respiratory failure requiring noninvasive positive pressure ventilation (NIPPV) in an adult.

CASE PRESENTATION: A 31 year old female presented with dyspnea and increasing lethargy. Past medical history is positive for left chest wall MCCL diagnosed at birth and underwent resection at age 2. Due to the reoccurrence of the large lymphangioma, the patient underwent several sclerotherapy treatments at age 10. Despite aggressive management, she had a left arm amputation and left breast reconstruction secondary to fibrosis. She had several recurrent hospitalizations for respiratory failure leading to unnecessary antibiotic treatment for suspected pneumonia. Vital signs were remarkable for tachycardia, tachypnea and hypoxia. Physical exam revealed decreased breath sounds on the left lung, a grade 2/6 systolic murmur and a large palpable mass in the posterior left thoracic wall. The ABG on 50% oxygen was: Ph 7.2/pCO2 88 /PO2 134. A chest CT scan showed an enlarging left posterior chest wall lymphangioma with significant atelectasis. Echocardiogram revealed severe pulmonary hypertension due to her chronic lung disease. The patient was immediately placed on NIPPV, which led to a quick improvement in her respiratory status.

DISCUSSION: Large MCCL of the chest wall can present as an atypical cause of acute severe respiratory distress. Workup includes imaging as well as tissue biopsy. The primary treatment is surgical excision. Alternative treatments for poor surgical candidates include sclerotherapy, chemotherapy, radiation, and laser therapy. In this case, the patient underwent surgical excision and sclerotherapy with no success. Therefore supportive treatments were implemented.

CONCLUSIONS: NIPPV should be considered in managing acute respiratory failure caused by chest wall MCCL as it provides lung recruitment and avoids the use of unnecessary antibiotics. Further multicenter studies to review the best treatment options and prognosis of such rare patient population are required.

Reference #1: Lee WS, et al. Cavernous Lymphangioma Arising in the Chest Wall 19 Years after Excision of a Cystic Hydroma. K. J Thorac Cardiovasc Surg 2011;44:380-382.

Reference #2: Krainick-Strobel U, et al. Massive Cavernous Lymphangioma of the breast and Thoracic Wall: Case Report and Literature Review. Lymphology 2006;39:147-151.

DISCLOSURE: The following authors have nothing to disclose: Omar Mousa, Vanessa Hoy, Andreea Nitu-Marquise, Matthew Hess

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