Postnatal Care and Sclerotherapy for Lymphatic Malformations

At The Children’s Hospital of Philadelphia, newborns with a neck mass are cared for in our Newborn/Infant Intensive Care Unit (N/IICU) by the Neonatal Surgical Team. The N/IICU’s core group of surgeons, neonatologists and specialized surgical nurse practitioners have partnered with the Vascular Anomalies Program, which includes caregivers from interventional radiology, general surgery, plastic surgery and ENT, for a comprehensive multidisciplinary approach, which is outlined in our Optimal Care Guidelines: Sclerotherapy for Lymphatic Malformations.

After admission to the N/IICU, the newborn is carefully assessed. Newborns asymptomatic at birth are followed closely clinically for oral feeding issues and positional stridor. Baseline postnatal MRI images are obtained. For those who are able to feed and sleep without symptoms, discharge with postnatal follow-up with the Vascular Anomalies team and general surgery occurs.

Sclerotherapy for lymphatic malformations

For those babies who are symptomatic, an ENT consult for airway evaluation occurs. An MRI of the neck and chest without contrast is obtained for treatment planning. Some newborns are candidates for surgical resection, others are treated with sclerotherapy alone, and some require a combination approach. A baseline hearing screen, total parenteral nutrition panel and complete blood count are obtained prior to initiation of sclerotherapy. Those newborns that undergo sclerotherapy remain intubated until sclerotherapy is completed.

There are different agents that may be used for sclerotherapy. One agent, doxycycline, irritates the inner epithelium in the cystic cavities and causes the inner walls to scar together. The cavity contracts and the volume drained impacts subsequent dosing. On the first day of treatment, drains are typically placed in interventional radiology and doxycycline is instilled for several hours. On days two and three of treatment, injections may be done at the bedside via pigtail catheter drains. Once sclerotherapy starts, the airway is automatically considered difficult or critical (per ENT). Doxycycline injections are generally very well tolerated. Side effects, including hypoglycemia and metabolic acidosis, can occur with high doses and typically occur within a few hours of treatment.

Other agents used are ethanol (ETOH) and sodium tetradecyl sulfate (STS). These agents are typically injected into the cysts, allowed to dwell for a period of several minutes, and then aspirated. STS is a detergent and causes transmembrane lipoproteins to be released from cell membranes allowing better penetration of ETOH into the cells. ETOH then denatures intracellular proteins causing cell death.

Typically STS and ETOH are used concurrently. Side effects of ETOH and STS are metabolic acidosis and can occur during dwell time. This is monitored clinically.

Infants must remain intubated for at least 48 hours following the last sclerotherapy treatment as this is when peak swelling occurs. Previously normal airways may now have significant airway swelling, and previously smaller cysts may now be larger as the cyst walls are altered by sclerotherapy. The degree of swelling is not predictable. A repeat MRI to evaluate the airway prior to extubation is performed and then an extubation plan is formulated.

Infants remain hospitalized until there are no signs of airway swelling (e.g., hoarseness, stridor) for at least 24 hours and should be making progress on oral feeds. A hearing test is repeated following sclerotherapy and prior to discharge. A car seat test is performed prior to discharge independent of gestational age. Infants younger than 6 months of age are discharged home on a pulse oximeter.

Patients are followed by general surgery and the Vascular Anomalies Program, led by Anne Marie Cahill, MD, chief of the Division of Interventional Radiology, and David Low, MD, attending surgeon in the Division of Plastic and Reconstructive Surgery. Audiology follow-up occurs at 6 months of age.

References

Cahill AM, Nijs E, Ballah D, et al. Percutaneous sclerotherapy in neonatal and infant head and neck lymphatic malformations: a single center experience. J Pediatr Surg. 2011;46(11):2083-2095. Jamal N, Ahmed S, Miller T, et al. Doxycycline  sclerotherapy for pediatric head and neck macrocystic lymphatic malformations: a case series and review of the literature. Int J Pediatr Otorhinolaryngol. 2012;76(8):1127-1131.

Laje P, Peranteau WH, Hedrick HL, Flake AW, Johnson MP, Moldenhauer JS, Adzick NS. Ex utero intrapartum treatment (EXIT) in the  management of cervical lymphatic malformation. J Pediatr Surg. 2015;50(2):311-314.

Laje P, Johnson MP, Howell LJ, Bebbington MW, Hedrick HL, Flake AW, Adzick NS. Ex utero intrapartum treatment in the management of giant cervical teratomas. J Pediatr Surg. 2012;47(6):1208-1216.

Laje P, Howell LJ, Johnson MP, Hedrick HL, Flake AW, Adzick NS. Perinatal management of congenital oropharyngeal tumors: the ex utero intrapartum treatment (EXIT) approach. J Pediatr Surg. 2013; 48(10):2005-2010.

Nehra D, Jacobson L, Barnes P, et al. Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children. J Pediatr Surg. 2008;43(3):451-460.