Isolated mediastinal lymphangioma in a child: A rare case report

SA Journal of Radiology, Jan 2025

The mediastinum is an unusual location for cystic lymphangiomas. An 18-month-old male presented with acute fever, dry intermittent cough and respiratory distress. Chest radiography, ultrasonography, contrast-enhanced CT and MRI suggested a diagnosis of mediastinal cystic lymphangioma with internal haemorrhage. Surgical excision of the lesion and histopathological examination confirmed cystic lymphangioma. CONTRIBUTION: This case highlights the multimodal radiological features of isolated mediastinal cystic lymphangioma for accurate diagnosis and improved management, to avoid unnecessary interventions and complications.Keywords : benign vascular tumour; mediastinal lymphangioma; cystic lymphangiomas; cystic lesion; recurrent pleural effusion.

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Isolated mediastinal lymphangioma in a child: A rare case report

SA Journal of Radiology ISSN: (Online) 2078-6778, (Print) 1027-202X Page 1 of 4 Case Report Isolated mediastinal lymphangioma in a child: A rare case report Authors: Ankita Gupta1 Maheswar Chaudhury1 Manoranjan Khuntia1 Peeta H. Prasad1 Somadatta Das1 The mediastinum is an unusual location for cystic lymphangiomas. An 18-month-old male presented with acute fever, dry intermittent cough and respiratory distress. Chest radiography, ultrasonography, contrast-enhanced CT and MRI suggested a diagnosis of mediastinal cystic lymphangioma with internal haemorrhage. Surgical excision of the lesion and histopathological examination confirmed cystic lymphangioma. Affiliations: 1 Department of Radiodiagnosis, Institute of Medical Sciences and SUM Hospital, Siksha ‘O’ Anusandhan, Deemed to be University, Bhubaneswar, India Contribution: This case highlights the multimodal radiological features of isolated mediastinal cystic lymphangioma for accurate diagnosis and improved management, to avoid unnecessary interventions and complications. Corresponding author: Manoranjan Khuntia, manoranjankhuntia@soa. ac.in Dates: Received: 09 Aug. 2025 Accepted: 16 Oct. 2025 Published: 20 Dec. 2025 How to cite this article: Gupta A, Chaudhury M, Khuntia M, Prasad PH, Das S. Isolated mediastinal lymphangioma in a child: A rare case report. S Afr J Rad. 2025;29(1), a3274. https://doi.org/10.4102/sajr. v29i1.3274 Copyright: © 2025. The Authors. Licensee: This work is licensed under the Creative Commons Attribution 4.0 International (CC BY 4.0) license (https:// creativecommons.org/ licenses/by/4.0/). Keywords: benign vascular tumour; mediastinal lymphangioma; cystic lymphangiomas; cystic lesion; recurrent pleural effusion. Introduction Solitary cystic lymphangioma is an uncommon congenital benign vascular tumour caused by a malformation of the lymphatic vessels. Lymphangiomas can affect any site in the body, most commonly the cervical (75%) and axillary regions (20%). Less than 1% of lymphangiomas are mediastinal.1 Most mediastinal lymphangiomas are located in the anterior mediastinum. Lymphangiomas, although benign, can present with complications such as infection, cystic haemorrhage, superior vena cava syndrome, airway compromise, chylothorax and chylopericardium.2 Landing and Farber classified lymphangiomas into three categories: • simple or capillary lymphangioma – dilated, capillary-sized lymphatic vessels connected to a normal lymphatic network • cystic lymphangioma – multiple large cyst-like spaces lined by flat endothelial cells; the cystic spaces may be empty or filled with clear proteinaceous or chylous fluid containing lymphocytes, or occasionally red blood cells • cavernous lymphangioma – dilated lymphatic sinuses in an actively growing lymphoid stroma, also connected to normal lymphatics.3,4 A compilation of clinical presentation, radiological imaging and histopathological investigation aids in diagnosing lymphangioma. A case of anterior mediastinal cystic lymphangioma with chief complaints of fever, intermittent cough and excessive crying in an 18-month-old male is presented. Ethical considerations Written informed consent was obtained from the legal guardian of the patient. Patient presentation Read online: Scan this QR code with your smart phone or mobile device to read online. An 18-month-old male was admitted to a tertiary care hospital for persistent fever and intermittent dry cough associated with excessive crying. He was delivered at term as the 1st order twin (the 2nd twin was stillborn), requiring admission to the neonatal intensive care unit for early-onset sepsis on day 2 of life, for which he received antibiotics and was discharged with no subsequent illness documented. On admission, physical examination revealed decreased air entry in the left mammary, infra-mammary and infra-axillary regions. The patient was febrile and irritable, with a per-abdominal examination suggesting hepatomegaly. An initial suspicion of meningitis was made, for which IV fluids and empiric antibiotics were commenced. Examination of the CSF revealed two white cells/µL, excluding meningitis. http://www.sajr.org.za Open Access Page 2 of 4 Chest radiography revealed a homogeneously opacified left hemithorax, silhouetting the ipsilateral cardiac margin and hemidiaphragm, and a widened mediastinum (Figure 1a). Ultrasound with Doppler of the thorax showed a welldefined, multiseptated, cystic lesion in the left hemithorax measuring 8 × 4 cm, abutting the pericardium (Figure 1b and Figure 1c). Contrast-enhanced CT (CECT) of the thorax with angiography was advised to exclude congenital pulmonary airway malformation (CPAM). Imaging with CT revealed a large, non-enhancing, septated, cystic lesion measuring 8.7 × 8.5 × 6.4 cm,without a perceptible wall, in the anterior mediastinum and left hemithorax (Figure 2a and Figure 2b). The mass extended to the superior mediastinum but did not involve the neck, abutting the right mediastinal pleura and pericardium and indenting the anterior wall of the thymus, causing secondary collapse of the left lung basal segments and deviation of the trachea to the right (Figure 2c and Figure 2d). The MRI showed a large T2 hyperintense cystic lesion with thin septations in the anterior mediastinum and left hemithorax compressing the left lung, consistent with the CECT findings (Figure 3). Dependent, non-enhancing, hyperdense content within the lesion on the CT scan corresponded to T1 hyperintensity on the MRI, likely indicating haemorrhage (Figure 4). Following a suggested diagnosis of mediastinal cystic lymphangioma complicated by internal haemorrhage, median sternotomy with excision of the anterior mediastinal cystic mass (Figure 5) was performed under general anaesthesia. Histopathological examination revealed fibroadipose tissue, cystic spaces lined by a corrugated, single layer of cuboidal epithelium and adjacent fibrovascular connective tissue showing dense lymphoplasmacytic infiltrates and scattered, dilated mature lymphocytes, a b Case Report suggesting a benign vascular malformation compatible with lymphangioma (Figure 6). The patient developed a Candida infection at the central line site on day 10 post-surgery, for which antifungals and antibiotics were administered. The patient was discharged on day 12 post-surgery. Discussion Cystic lymphangiomas most commonly develop superficially on the body surface and are often detected before the age of 2 years.1,5 Only 2% – 3% of the cervical lymphangiomas may be associated with an intrathoracic extension. An isolated mediastinal lymphangioma without a cervical component is rare, accounting for less than 1%.5 The child in the presented case had an isolated mediastinal cystic lymphangioma without any cervical component. Cystic lymphangiomas are usually asymptomatic until they reach dimensions large enough to cause compression of the a b c d FIGURE 2: (a, b) Contrast-enhanced CT (CECT) revealing a large, no (...truncated)


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Ankita Gupta, Maheswar Chaudhury, Manoranjan Khuntia, Peeta H. Prasad, Somadatta Das. Isolated mediastinal lymphangioma in a child: A rare case report, SA Journal of Radiology, 2025, pp. 1-4, Volume 29, Issue 1, DOI: 10.4102/sajr.v29i1.3274