1 - Department of Pediatric Surgery, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal
2 - Unit of Neonatal Intensive Care, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal
3 - Department of Pediatrics, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal
4 - Department of Dermatology, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal
- III Congresso Nacional de Anomalias Vasculares
- Porto, 24 de Março de 2023
Introduction: Vascular malformations are a broad spectrum of congenital lesions, involving vascular structures. The lymphatic type is composed of anomalous lymphatic vessels resulting in abnormal lymphatic flow. Normally they present at birth or within the first few years of life. Imaging is important for diagnosis and condition’s assessment. Prenatal diagnosis is possible by ultrasonography, in the second and third trimesters. Not all lymphatic malformations (LM) need intervention, but, according to clinical implications, most cases require a multidisciplinary approach.
Clinical case: A forty week gestation male, with prenatal diagnosis of a large head and neck LM, during childbirth had rupture of the lesion with subsequent aponeurosis and muscle exposure of temporal region. Regarding the prenatal period, the second trimester ultrasound diagnosed a multiseptated cystic lesion in left side of head and face, with extension to cervical area suggesting a LM. Fetal MRI was performed to better evaluate this lesion, describing it as large and multiseptated extending to the subcutaneous compartment of the left side of face and head, compatible with LM; there was no involvement of intracranial structures; cervical component without involvement of the airway or vascular structures; face component with extension to the left palpebral plane, but independent of the orbital cavity. On 5th day of life the patient underwent surgical treatment, with biopsies of skin and lymphatic lesion, partial excision of cystic walls, reposition of pinna and skin closure with local flaps.; no complications registered. The anatomopathological report was compatible with LM. Currently, the patient is under treatment with Sirolimus and has been followed by an interdisciplinary team, composed by Pediatric Plastic Surgery, Hematology, Dermatology and Immunodeficiencies.
Discussion: In prenatally diagnosed LM, imaging is essential to evaluate the lesion, but also for planning the delivery procedure. Postnatal surgical excision is the treatment of choice for small well-localized lesions, but involvement of the airway or other essential structures can interfere with the resection’s success rate.
Conclusion: Managing of LM with prenatal diagnosis depends of lesion’s characteristics and should be planned with an interdisciplinary team in order to decrease peripartum morbility and achieve better treatment results.
Palavras Chave: lymphatic malformation , prenatal