Management of an Infant with Complex Cystic Hygroma of the Neck

Cystic hygromas are fluid-filled sacs that result from a blockage in the lymphatic  system.  Cystic hygromas can be single or multiple cysts found mostly in the neck region.  Cystic hygromas occur in approximately 1% of fetuses between 9 and 16 weeks of pregnancy (Oldham et.al, 1998).  Environmental causes for cystic hygromas include maternal viral infections such as Parvovirus  Fifth’s disease.  Genetic syndromes associated with cystic hygroma are Turner Syndrome, Trisomies such as 13,18, and 21(Emory University, Department of Genetics, 2008).  Enlargement of the cystic hygroma progresses with a  child’s growth. They can have a sudden increase in size secondary to intralesional bleeding.  Spontaneous decompression or shrinkage is uncommon.   Cystic hygromas tend to predominate below the mylohyoid muscle and can involve both the anterior and posterior triangles of the neck.  This poster describes a case study of a 7month old infant who was admitted to Johns Hopkins Hospital at one month of age for evaluation and management of a large cystic hygroma of her neck/face/mediastinum.   The baby was born at 38 weeks gestation from the UAE.  Upon arrival to JHH, her respiratory effort was poor and a tracheostomy was performed emergently. A stage II pressure ulcer developed from the tracheostomy tubing.   In addition, a bilateral modified radical neck dissection with extension to left posterior triangle and suboccipital region of the mass was excised.   Several days later a portion of the wound dehisced.  Both wounds were treated with silver alginate and slowly healed.   Once stable, Interventional radiology and ENT coordinated therapy to include sclerosing the area with Doxycycline.   Sclerotherapy is effective in shrinking macrocystic lesions.  These lesions are channels measuring 2cm or greater.  However, cysts measuring as small as 5mm have been able to be sclerosed with ultrasonic guidance.    Approximately five days later the baby’s Hgb dropped and the hygroma appeared to increase in size acutely.  Patient  had six drains placed by CVDL to aid in release of fluid collections.  A study on sclerosing/laser of cystic hygromas found they have a tendency to recur often after treatment (Oldham, et al, 1998).    Consequently, most feel resection is the best cure.  However, complete resection is often challenging because the lesion permeates tissue, nerves, and muscles. Staged excision is usually necessary. (Lacey SR, et al, 1988).

After several therapies it was decided that the therapy had been exhausted and she would return home for additional sclerosising treatment.   Unfortunately, after several successful sclerosing treatments she contracted H1N1 and was unable to overcome the virus which cause her to fall to her demise.

Treatment goals included: 1. Constant monitoring of the face/neck area involved with the hygroma for skin breakdown.  The large hygroma was subject to sheering and friction increasing chances of skin breakdown.  2.  Evaluate effectiveness of therapy.  3.  Evaluate need for therapy changes.

Results of this case study reveal the need for education of wound care nurses and hospital staff for complex pediatric wounds.  In-services and educational courses can be targeted to meet the needs of the WOCN nurses and involved nursing staff.