Multispecialty Approach to a Very Large Congenital Head and Neck Cystic Lymphatic Malformation in an Infant Born by SARS-CoV-2 Positive Mother—A Case Report

Masses of the head and neck are often diagnosed prenatally and require special care due to the risk of airway obstruction. The EXIT procedure is a preferable mode of delivery. A congenital cystic lymphatic malformation is one of the most common lesions of the cervical region described in neonates. The treatment consists of different strategies and involves the cooperation of multiple specialists. Up to now, no guidelines or protocols are available. We report a case of a congenital cystic lymphatic malformation of the head and neck delivered during the EXIT procedure by a mother who was SARS-CoV-2 positive. We analyzed clinical characteristics, radiologic features, and treatment with injections of sclerotic agents and orally administrated sirolimus. Sirolimus seems a valuable and safe therapeutic option for treating lymphatic malformations, especially with adjunct therapies.

[1]  Tatsuya Suzuki,et al.  A neonatal large neck lymphatic malformation successfully treated with Eppikajyutsuto , 2022, Journal of Pediatric Surgery Case Reports.

[2]  D. Song,et al.  Refractory Head and Neck Lymphatic Malformation in Infants Treated With Sirolimus: A Case Series , 2021, Frontiers in Oncology.

[3]  G. Antiñolo,et al.  Ex-Utero Intrapartum Treatment (EXIT): indications and outcome in fetal cervical and oropharyngeal masses , 2020, BMC Pregnancy and Childbirth.

[4]  J. L. López Gutiérrez,et al.  Oral Sirolimus: An Option in the Management of Neonates with Life-Threatening Upper Airway Lymphatic Malformations. , 2019, Lymphatic research and biology.

[5]  M. Rajewski,et al.  Use of ex utero intrapartum treatment procedure in fetal neck and high airway anomalies – report of four clinical cases , 2019, The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians.

[6]  C. Mounayer,et al.  Head and neck lymphatic malformation and treatment: Clinical study of 23 cases. , 2016, European annals of otorhinolaryngology, head and neck diseases.

[7]  J. López-Gutiérrez,et al.  Sirolimus in the Treatment of Vascular Anomalies , 2016, European Journal of Pediatric Surgery.

[8]  Haneen T Salah,et al.  Treatment of cystic hygroma in a young infant through multidisciplinary approach involving sirolimus, sclerotherapy, and debulking surgery , 2016, JAAD case reports.

[9]  S. Mccarroll,et al.  Lymphatic and other vascular malformative/overgrowth disorders are caused by somatic mutations in PIK3CA. , 2015, The Journal of pediatrics.

[10]  A. Flake,et al.  Ex utero intrapartum treatment (EXIT) in the management of cervical lymphatic malformation. , 2015, Journal of pediatric surgery.

[11]  S. Gaddikeri,et al.  Congenital cystic neck masses: embryology and imaging appearances, with clinicopathological correlation. , 2014, Current problems in diagnostic radiology.

[12]  S. Beasley,et al.  The ex utero intrapartum treatment (EXIT) procedure: Application of a new therapeutic paradigm , 2013, Journal of paediatrics and child health.

[13]  J. Moldenhauer Ex Utero Intrapartum Therapy. , 2013, Seminars in pediatric surgery.

[14]  D. Ballah,et al.  Percutaneous sclerotherapy in neonatal and infant head and neck lymphatic malformations: a single center experience. , 2011, Journal of pediatric surgery.

[15]  M. Ibrahim,et al.  Congenital cystic lesions of the head and neck. , 2011, Neuroimaging clinics of North America.

[16]  Muhammad Sharif,et al.  Cystic Hygroma: An Overview , 2010, Journal of cutaneous and aesthetic surgery.

[17]  Yutaka Sato,et al.  Efficacy and safety of OK‐432 immunotherapy of lymphatic malformations , 2009, The Laryngoscope.

[18]  P. Barnes,et al.  Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children. , 2008, Journal of pediatric surgery.

[19]  D. Waldman,et al.  Sclerotherapy with use of doxycycline after percutaneous drainage of postoperative lymphoceles. , 2001, Journal of vascular and interventional radiology : JVIR.

[20]  D. Poenaru,et al.  Impact of magnetic resonance imaging on the surgical management of cystic hygromas. , 1998, Journal of pediatric surgery.

[21]  E. Deguchi,et al.  OK-432 therapy for lymphangioma in children: why and how does it work? , 1996, Journal of pediatric surgery.

[22]  G. Enríquez,et al.  US, CT, and MR imaging of neck lesions in children. , 1995, Radiographics : a review publication of the Radiological Society of North America, Inc.

[23]  M. Mafee,et al.  Congenital malformations of the cervicothoracic lymphatic system: embryology and pathogenesis. , 1992, Radiographics : a review publication of the Radiological Society of North America, Inc.

[24]  W. Smoker,et al.  The anterior and posterior cervical spaces. , 1991, Seminars in ultrasound, CT, and MR.