Refractory chylothorax and chylous ascites as form of presentation of diffuse lymphangiomatosis

Láinez Ramos-Bossini, Antonio Jesús; Ruiz-Carazo, Eduardo; Gálvez López, Regina

doi:10.1016/j.medcle.2020.03.017

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Once it was established that she had high blood pressure, she was admitted for foetal monitoring. The patient had a normal delivery on the third day. Given the persistence of dyspnoea, a computed tomography (CT) angiography was performed which ruled out pulmonary thromboembolism, but a large bilateral pleural effusion, ascites and multiple simple splenic cysts were observed. A pleural fluid sample was collected, showing a milky appearance suggestive of chylothorax. A biochemical analysis confirmed the suspicion (>1,700 mg/dL of triglycerides), and microbiological studies (including mycobacteria), thyroid hormones and a bone marrow aspirate showed no significant abnormalities.A NPO (nil per os) diet was initiated, with total parenteral nutrition. Treatment with somatostatin was also prescribed, but it was discontinued due to poor tolerance. Given the recurrence of the chylothorax, a thoracic duct ligation was performed, which proved ineffective, so a lymphoscintigraphy and SPECT-CT were requested, demonstrating an abnormal deposit of a radiopharmaceutical in the lesser sac, showing several images compatible with lymphatic cysts. A laparotomy was performed with biopsy and removal of the spleen and lymphatic lesions, the histological analysis of which revealed multiple lymphangiomas. After surgery, the patient improved and was discharged. However, a few months later she went to the emergency room due to recurrence of chylous ascites and chylothorax, opting for admission to the Internal Medicine service with implantation of a peritoneal catheter. However, she had multiple episodes of spontaneous bacterial peritonitis and a torpid course. Two months later, she experienced multi-organ failure and eventually died.The clinical presentation of lymphangiomatosis varies according to the anatomical area involved.<a class="elsevierStyleCrossRef" href="#bib0010">2</a> Pulmonary involvement usually causes cough, dyspnoea, chest pain, or chylothorax; in the abdomen it can manifest as nonspecific pain, nausea, vomiting, chylous ascites or intestinal bleeding; and bone involvement can cause pain, pathological fractures, and joint deformity.<a class="elsevierStyleCrossRefs" href="#bib0005">1–3</a> Due to the low specificity of symptoms, patients with lymphangiomatosis are susceptible to misdiagnoses and delays. The established diagnosis must be made through anatomical pathology. Histologically, lymphangiomas consist of endothelial-lined cystic spaces that contain homogeneous eosinophilic material and are surrounded by a connective tissue stroma with lymphatic tissue, rounded cells, and smooth muscle.<a class="elsevierStyleCrossRefs" href="#bib0010">2,3</a> Because taking biopsies is not always possible, characterization using non-invasive imaging tests (CT or MRI) is gaining more and more prominence. Lymphangiography is the technique of choice in the presence of chylous leak or fistula, chylothorax, chylous ascites, or chyluria, as it allows determining the location, size, and distribution of lymphatic lesions, and may also have therapeutic value.<a class="elsevierStyleCrossRef" href="#bib0020">4</a> Treatment is variable and depends on the location, extent, and severity. In asymptomatic cases, a watchful waiting approach is preferable, while in symptomatic patients, non-invasive options (low-fat diet, total parenteral nutrition, corticosteroids, bisphosphonates, sirolimus, thalidomide, interferon, cyclophosphamide, tamoxifen, sildenafil) and invasive options (repeat pleurodesis, pleurectomy, thoracentesis, sclerotherapy, percutaneous embolization, or radiation therapy).<a class="elsevierStyleCrossRefs" href="#bib0010">2,4</a>In conclusion, lymphangiomatosis is a rare entity with a difficult diagnosis and a potentially fatal prognosis. Due to its implications, it is necessary to consider this entity in the differential diagnosis of ascites and chylous pleural effusion resistant to treatment.FundingNo funding was received for this paper.Conflict of interestsThe authors declare that they have no conflicts of interest." 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Med Clin (Barc). 2021;156:584." ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinical and radiological features of generalised lymphangiomatosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "C. Wong" 1 => "T. Chu" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Hong Kong Med J" "fecha" => "2008" "volumen" => "14" "paginaInicial" => "402" "paginaFinal" => "404" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/18840914" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Abdominal lymphangiomatosis in a 38-year-old female: case report and literature review" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "R.Y. Lin" 1 => "Z. Hai" 2 => "C. Tan Zhou" 3 => "W. Wei" 4 => "W. Jiang-Hong" 5 => "H. Qing-Xi" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3748/wjg.v20.i25.8320" "Revista" => array:6 [ "tituloSerie" => "World J Gastroenterol" "fecha" => "2014" "volumen" => "20" "paginaInicial" => "8320" "paginaFinal" => "8324" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25009412" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Lymphangiomatosis in a 14-year-old female presenting with chylothorax and multiple cystic lesions" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "R. Uribe" 1 => "S. Isaza" 2 => "V. Prada" 3 => "L. Cadavid" 4 => "W. Quiceno" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.radcr.2018.05.002" "Revista" => array:6 [ "tituloSerie" => "Radiol Case Rep" "fecha" => "2018" "volumen" => "13" "paginaInicial" => "782" "paginaFinal" => "787" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/30002781" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The effectiveness of lymphangiography as a treatment method for various chyle leakages" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "T. Matsumoto" 1 => "T. Yamagami" 2 => "T. Kato" 3 => "T. Hirota" 4 => "R. Yoshimatsu" 5 => "T. Masunami" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1259/bjr/64849421" "Revista" => array:6 [ "tituloSerie" => "Br J Radiol" "fecha" => "2009" "volumen" => "82" "paginaInicial" => "286" "paginaFinal" => "290" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19029221" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015600000011/v1_202106060546/S2387020621002187/v1_202106060546/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015600000011/v1_202106060546/S2387020621002187/v1_202106060546/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020621002187?idApp=UINPBA00004N"]

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