P456 Management of cystic lymphangiomas in the newborn: a study of 6 cases. (June 2019)

• Record Type: Journal Article
• Authors: Charfi, Manel; Zaghdoud, Rim; Regaieg, Chiraz; Hmed, Amel Ben; Bouraoui, Amira; Regaieg, Ridha; Hmida, Nedia; Thabet, Afef Ben; Gargouri, Abdellatif
• Is Part Of: Archives of disease in childhood. Volume 104:(2019)Supplement 3
• Page Start: A335
• Page End: A335
• Publication Date: 2019-06
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2019-epa.792
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Background: Cystic lymphangiomas are rare congenital benign malformations of the lymphatic system. They are commonly located in the cervicofacial region. Clinical presentation depends on localization. Through this study we aim to identify epidemiology, clinical features, management and outcome of cystic lymphangiomas in the newborn. Methods: It's a retrospective study of all cases of cystic lymphangiomas registered in the neonatal intensive care unit of Sfax between 2008 and 2018. Results: Six full term newborns were included with a female:male ratio of 4:2. Prenatal diagnosis was performed in all cases. At birth all newborns was asymptomatic. Post natal diagnosis was based on the ultrasound study in all cases. Magnetic resonance imaging was performed in three cases. We registered cervical localization in three cases, cervicomediastinal localization in one case, carvicomediastinal localization associated to multiple lesions of the right arm in one case and perirenal localization in one case. The mean size of masses was 8.6 cm (5 to 15 cm). Total resection was performed successfully for the perirenal lymphangioma at day 22 of life. In the post operative course the newborn developed a transient hypertension and an urinoma that regressed after 10 days of drainage. Incomplete resection was performed in one newborn having cervicomediastinal localization with close relation to the atrium leading to severe respiratory distress at day 4 of life. The patient died of severe sepsis on the post operative course. Embolization was proposed for two newborns but they rapidly died. For the two other cases we opted for monitoring. Spontaneous regression was then noted. No recurrence was registered in all surviving newborns. Conclusions: Cystic lymphangiomas can arise in any organ or soft tissue. Mediastinal and abdominal localization are rare. Management and outcome depends on localization and relationship with adjacent structures. Less invasive therapeutic option and new therapies continue to emerge. However till now there are not uniform therapeutic protocols.