Late diagnosis of generalized lymphangiomatosis in a woman presenting with respiratory distress

Mehrnahad, Mersad; Valeshabad, Ali Kord; Rezaei, Zahra; Kord, Reza

doi:10.1016/j.radcr.2020.05.021

Cited by 6 publications

(3 citation statements)

References 21 publications

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“…A recent review of 35 cases of cardiac or pericardiac cystic lymphangioma revealed that eight patients were asymptomatic and were diagnosed incidentally. The sudden appearance of symptoms of lymphangiomas is generally attributed to rapid enlargement of an existing lesion due to inflammation, hemorrhage, or trauma [ 11 ]. In our patient, the sudden onset of respiratory distress was most likely due to an intracystic hemorrhage.…”

Section: Discussionmentioning

confidence: 99%

A large pericardial cystic lymphangioma presenting as acute-onset respiratory distress in a child: a case report

et al. 2022

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Background Lymphangiomas are rare benign malformations of the lymphatics that occur due to blockage of the lymphatic system during fetal development. They commonly occur in the neck and axilla, while involvement of the pericardium is rare. We report herein the case of a 16-month-old Sri Lankan child with a large pericardial cystic lymphangioma presenting with sudden-onset shortness of breath. Case presentation A 16-month-old Sri Lankan boy presented with sudden-onset dyspnea for 1-day duration following a febrile illness that lasted 2 days. On examination, he was afebrile and had subcostal, intercostal, and suprasternal recessions, with a respiratory rate of 50 breaths per minute. He had a loud expiratory grunt. The chest expansion was reduced on the right side, which was dull to percussion. Auscultation revealed a marked reduction of air entry over the right lower and mid zones. Chest X-ray showed a well-demarcated opacity involving the lower and mid zones of the right hemithorax associated with a tracheal shift to the opposite side. Ultrasound scan of the chest revealed fluid-filled right hemithorax suggesting a septate pleural effusion. A contrast-enhanced computed tomography scan of the thorax showed a large multiloculated extrapulmonary cystic lesion involving the right hemithorax with a mediastinal shift towards the left side associated with displacement of the right-side mediastinal structures. He underwent mini-thoracotomy and surgical excision of the cyst. A large cyst originating from the pericardium was observed and excised during surgery. Histological examination revealed a lesion composed of cysts devoid of a lining epithelium but separated by connective tissue, mature adipose tissue, and lymphoid aggregates. The child showed complete recovery postoperatively with full expansion of the ipsilateral lung. Conclusion We report the case of a patient with cystic lymphangioma who was perfectly well and asymptomatic until 16 months of age. This case report presents the very rare occurrence of a large cystic lymphangioma originating from the pericardium. It highlights the importance of considering rare possibilities and performing prompt imaging in situations of diagnostic uncertainty to arrive at an accurate diagnosis that can be lifesaving.

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Section: Discussionmentioning

confidence: 99%

A large pericardial cystic lymphangioma presenting as acute-onset respiratory distress in a child: a case report

et al. 2022

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“…They can originate from any tissue except ocular and neural tissue that can involve multiple organs with a variety of clinical presentations. This condition may involve soft tissues, bone structures, and various organs in the body such as the liver, spleen, mediastinum, and lungs [1] , [2] , [3] . Despite children, generalized cystic lymphangiomatosis is extremely rare in adults [4] .…”

Section: Introductionmentioning

confidence: 99%

“…This condition occurs with an equal gender prevalence [2] . We report a very rare case of incidentally diagnosed generalized lymphangiomatosis involving the liver, spleen, mediastinum, vertebrae, and iliac bones in a 42-year-old male, which was subsequently confirmed by histological findings.…”

Section: Introductionmentioning

confidence: 99%

Generalized cystic lymphangiomatosis incidentally diagnosed in an asymptomatic adult: Imaging findings of a very rare case

Tabrizi,

Dadkhah,

soleimani

et al. 2024

Radiology Case Reports

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Generalized lymphangiomatosis in patients treated for chylothorax following thoracoscopic sympathectomy: Case report

Tuan,

Hien,

Trinh

et al. 2024

Radiology Case Reports

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Late diagnosis of generalized lymphangiomatosis in a woman presenting with respiratory distress

Cited by 6 publications

References 21 publications

A large pericardial cystic lymphangioma presenting as acute-onset respiratory distress in a child: a case report

A large pericardial cystic lymphangioma presenting as acute-onset respiratory distress in a child: a case report

Generalized cystic lymphangiomatosis incidentally diagnosed in an asymptomatic adult: Imaging findings of a very rare case

Generalized lymphangiomatosis in patients treated for chylothorax following thoracoscopic sympathectomy: Case report

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