Lymphatic Malformation of Tongue in A Newborn

Clinical History

A 21 year-old woman presented with a 27 week-old, single viable fetus. A multicystic echogenic giant mass which infiltrated the tongue and fulfilled the oral cavity was demonstrated on the ultrasound. Fetal MR imaging confirmed the diagnosis. The lesion shrunk after sclerotherapy. The baby died due to pneumonia after one month.

Imaging Findings

Figure 1 (a-f): On prenatal ultrasound (27 weeks) a giant LM of tongue with micro (arrow) and macrocytic (stars) cervical components is demonstrated (a,b). Macroglossia is shown on 4D image (c). Fetal MRI (27 weeks) shows that a microcystic LM of tongue with macrocystic cervical components (arrows) with open-upper airway on sagittal and coronal images (d-f).
Figure 2 (a, b): Preoperative photo image of the mass (a), the mass has regressed after the sclerotherapy (b).

Discussion

Lymphatic malformations (LMs) are composed of small or large cystic spaces [1]. LM is most frequently diagnosed in the head and neck, but can present rarely in mediastinum, abdomen and extremities. Many cases are diagnosed through obstetrical US and confirmed after birth. They are categorized in three groups: macrocystic, microcystic (< 2 mm) and mixed. On ultrasound, macrocystic lesions are anechoic multicystic-multiseptated masses with fluid-fluid levels whereas microcystic lesions have more cellular matrix which show hyperechogenicity. On Colour Doppler ultrasound, the septae do not have any flow within [2]. Microcystic lesions infiltrate and then enlarge a body part, tissue or organ. With the help of prenatal ultrasound and fetal MRI, airway safety and planning the ex utero intrapartum treatment procedure (EXIT) are possible. EXIT procedure with oropharyngeal intubation was performed on our patient by displacing the mass to a side successfully. Sclerotherapy is the primary treatment method to obliterate the cystic spaces and cause fibrosis of the lesion [3]. Prenatal diagnosis of LM requires fetal and postnatal MRI in order to delineate the anatomical extent of the lesion. Fetal MRI is the key imaging modality to show airway anatomy and plan EXIT procedure in case of potential airway obstruction by LM. Sclerotherapy is the primary treatment method with a high satisfaction in lesion regression. In addition, a multidisciplinary approach should be carried out in order to allow a better understanding in prenatal diagnosis and management of LMs.

Differential Diagnosis List

Final Diagnosis

Lymphatic Malformation of Tongue

URL:	https://www.eurorad.org/case/12395
DOI:	10.1594/EURORAD/CASE.12395
ISSN:	1563-4086